OtoWiki - User contributions [en]

Carotid-Cavernous Fistula

2025-11-22T18:53:52Z

Jack.Dewey: /* Classification */

{{infobox Disease
|Title =
|Aliases = Caroticocavernous Fistula
|Image = [Type D caroticocavernous fistula from left internal and external carotid arteries.png]
|Caption = A Barrow Type D carotid-cavernous fistula on CT angiography
|ICD-9 = 437.3
|ICD-10 = I67.1
|MeSH =
|Gene =
|Locus =
|OMIM =
|EyeWiki = [https://eyewiki.org/Carotid_Cavernous_Fistula Carotid Cavernous Fistula]
|Radiopaedia = [https://radiopaedia.org/articles/caroticocavernous-fistula-1?lang=us Carotiocavernous Fistula]
|Pathology =
}}

== Overview ==
'''Carotid-cavernous fistula''', or '''caroticocavernous fistula''', is an acquired communication between the carotid artery system and the cavernous sinus.

== Pathophysiology ==
=== Relevant Anatomy ===
<gallery>
Gray571.png|Contents of the cavernous sinus
Anatomy of the cavernous sinus.jpg|Bilateral cavernous sinus contents with communication through the circular venous plexus
Sobo 1909 589.png|Lateral view of the cavernous sinus in the skull base
Cavernous sinus.png|Axial view of the cavernous sinus
</gallery>

=== Disease Etiology ===

=== Classification ===
Broadly, carotid-cavernous fistulas can be characterized as either ''direct'' or ''indirect''. ''Direct'' fistulas have a direct communication between the intracavernous ICA itself and the cavernous sinus. ''Indirect'' fistulas describe communications from the carotid system to the cavernous sinus through a third vessel, a branch of the ICA or ECA. The most common classification system for carotid-cavernous fistulas is the Barrow classification system, first described in 1985.<ref>Barrow DL, Spector RH, Braun IF, Landman JA, Tindall SC, Tindall GT. Classification and treatment of spontaneous carotid-cavernous sinus fistulas. Journal of neurosurgery. 1985 Feb 1;62(2):248-56. DOI: https://doi.org/10.3171/jns.1985.62.2.0248 </ref>

{| class="wikitable", style="margin-left: auto; margin-right: auto; border: none; text-align: center"
|+ Barrow Classification for Carotid-Cavernous Fistula
|-
! Class !! Communication !! Description
|-
| A || Direct || Flow from the intracavernous ICA directly into the cavernous sinus
|-
| B || Indirect || Flow from a branch of the intracavernous ICA into the cavernous sinus via a dural shunt
|-
| C || Indirect || Flow from a meningeal branch of the ECA into the cavernous sinus via a dural shunt
|-
| D || Indirect || Multiple dural shunts flowing into the cavernous sinus from the ICA and ECA systems (Class B + C)
|}

Additional descriptive terms used to classify or categorize carotid-cavernous fistulas are similar to other arteriovenous fistulas, including high flow vs low flow and underlying etiology.

== Diagnosis ==
=== Patient History ===
=== Physical Examination ===
=== Laboratory Tests ===
=== Imaging ===
CT angiography or MR angiography are typically the first line imaging modality based on patient presentation and ease of availability in the emergency department. Angiography is the gold standard of diagnostic imaging in order to confirm the communication, as well as describe the location and flow of the fistula.

=== Differential Diagnosis ===

== Management ==
=== Medical Management ===
=== Surgical Management ===

== Outcomes ==
=== Complications ===
=== Prognosis ===

== References ==
<references />

Carotid-Cavernous Fistula

2025-11-22T18:49:49Z

Jack.Dewey: /* Classification */

{{infobox Disease
|Title =
|Aliases = Caroticocavernous Fistula
|Image = [Type D caroticocavernous fistula from left internal and external carotid arteries.png]
|Caption = A Barrow Type D carotid-cavernous fistula on CT angiography
|ICD-9 = 437.3
|ICD-10 = I67.1
|MeSH =
|Gene =
|Locus =
|OMIM =
|EyeWiki = [https://eyewiki.org/Carotid_Cavernous_Fistula Carotid Cavernous Fistula]
|Radiopaedia = [https://radiopaedia.org/articles/caroticocavernous-fistula-1?lang=us Carotiocavernous Fistula]
|Pathology =
}}

== Overview ==
'''Carotid-cavernous fistula''', or '''caroticocavernous fistula''', is an acquired communication between the carotid artery system and the cavernous sinus.

== Pathophysiology ==
=== Relevant Anatomy ===
<gallery>
Gray571.png|Contents of the cavernous sinus
Anatomy of the cavernous sinus.jpg|Bilateral cavernous sinus contents with communication through the circular venous plexus
Sobo 1909 589.png|Lateral view of the cavernous sinus in the skull base
Cavernous sinus.png|Axial view of the cavernous sinus
</gallery>

=== Disease Etiology ===

=== Classification ===
Broadly, carotid-cavernous fistulas can be characterized as either ''direct'' or ''indirect''. ''Direct'' fistulas have a direct communication between the intracavernous ICA itself and the cavernous sinus. ''Indirect'' fistulas describe communications from the carotid system to the cavernous sinus through a third vessel, a branch of the ICA or ECA. The most common classification system for carotid-cavernous fistulas is the Barrow classification system, first described in 1985.<ref>Barrow DL, Spector RH, Braun IF, Landman JA, Tindall SC, Tindall GT. Classification and treatment of spontaneous carotid-cavernous sinus fistulas. Journal of neurosurgery. 1985 Feb 1;62(2):248-56. DOI: https://doi.org/10.3171/jns.1985.62.2.0248 </ref>

{| class="wikitable", style="margin-left: auto; margin-right: auto; border: none; text-align: center"
|+ Barrow Classification for Carotid-Cavernous Fistula
|-
! Class !! Communication !! Description
|-
| A || Direct || Flow from the intracavernous ICA directly into the cavernous sinus
|-
| B || Indirect || Flow from a branch of the intracavernous ICA into the cavernous sinus via a dural shunt
|-
| C || Indirect || Flow from a meningeal branch of the ECA into the cavernous sinus via a dural shunt
|-
| D || Indirect || Multiple dural shunts flowing into the cavernous sinus from the ICA and ECA systems (Class B + C)
|}

== Diagnosis ==
=== Patient History ===
=== Physical Examination ===
=== Laboratory Tests ===
=== Imaging ===
CT angiography or MR angiography are typically the first line imaging modality based on patient presentation and ease of availability in the emergency department. Angiography is the gold standard of diagnostic imaging in order to confirm the communication, as well as describe the location and flow of the fistula.

=== Differential Diagnosis ===

== Management ==
=== Medical Management ===
=== Surgical Management ===

== Outcomes ==
=== Complications ===
=== Prognosis ===

== References ==
<references />

Carotid-Cavernous Fistula

2025-11-22T18:47:34Z

Jack.Dewey: /* Pathophysiology */

{{infobox Disease
|Title =
|Aliases = Caroticocavernous Fistula
|Image = [Type D caroticocavernous fistula from left internal and external carotid arteries.png]
|Caption = A Barrow Type D carotid-cavernous fistula on CT angiography
|ICD-9 = 437.3
|ICD-10 = I67.1
|MeSH =
|Gene =
|Locus =
|OMIM =
|EyeWiki = [https://eyewiki.org/Carotid_Cavernous_Fistula Carotid Cavernous Fistula]
|Radiopaedia = [https://radiopaedia.org/articles/caroticocavernous-fistula-1?lang=us Carotiocavernous Fistula]
|Pathology =
}}

== Overview ==
'''Carotid-cavernous fistula''', or '''caroticocavernous fistula''', is an acquired communication between the carotid artery system and the cavernous sinus.

== Pathophysiology ==
=== Relevant Anatomy ===
<gallery>
Gray571.png|Contents of the cavernous sinus
Anatomy of the cavernous sinus.jpg|Bilateral cavernous sinus contents with communication through the circular venous plexus
Sobo 1909 589.png|Lateral view of the cavernous sinus in the skull base
Cavernous sinus.png|Axial view of the cavernous sinus
</gallery>

=== Disease Etiology ===

=== Classification ===
Broadly, carotid-cavernous fistulas can be characterized as either ''direct'' or ''indirect''. ''Direct'' fistulas have a direct communication between the intracavernous ICA itself and the cavernous sinus. ''Indirect'' fistulas describe communications from the carotid system to the cavernous sinus through a third vessel, a branch of the ICA or ECA. The most common classification system for carotid-cavernous fistulas is the Barrow classification system, first described in 1985.

{| class="wikitable", style="margin-left: auto; margin-right: auto; border: none; text-align: center"
|+ Barrow Classification for Carotid-Cavernous Fistula
|-
! Class !! Communication !! Description
|-
| A || Direct || Flow from the intracavernous ICA directly into the cavernous sinus
|-
| B || Indirect || Flow from a branch of the intracavernous ICA into the cavernous sinus via a dural shunt
|-
| C || Indirect || Flow from a meningeal branch of the ECA into the cavernous sinus via a dural shunt
|-
| D || Indirect || Multiple dural shunts flowing into the cavernous sinus from the ICA and ECA systems (Class B + C)
|}

== Diagnosis ==
=== Patient History ===
=== Physical Examination ===
=== Laboratory Tests ===
=== Imaging ===
CT angiography or MR angiography are typically the first line imaging modality based on patient presentation and ease of availability in the emergency department. Angiography is the gold standard of diagnostic imaging in order to confirm the communication, as well as describe the location and flow of the fistula.

=== Differential Diagnosis ===

== Management ==
=== Medical Management ===
=== Surgical Management ===

== Outcomes ==
=== Complications ===
=== Prognosis ===

== References ==
<references />

Carotid-Cavernous Fistula

2025-11-22T18:23:34Z

Jack.Dewey: /* Imaging */

Carotid-Cavernous Fistula

2025-11-21T17:59:57Z

Jack.Dewey:

Carotid-Cavernous Fistula

2025-11-21T12:18:44Z

Jack.Dewey: /* Relevant Anatomy */

Carotid-Cavernous Fistula

2025-11-21T12:11:48Z

Jack.Dewey: /* Relevant Anatomy */

Carotid-Cavernous Fistula

2025-11-17T03:26:12Z

Jack.Dewey: Created page with "{{infobox Disease |Title = |Aliases = Caroticocavernous Fistula |Image = [Type D caroticocavernous fistula from left internal and external carotid arteries.png] |Caption = A Barrow Type D carotid-cavernous fistula on CT angiography |ICD-9 = 437.3 |ICD-10 = I67.1 |MeSH = |Gene = |Locus = |OMIM = |EyeWiki = [https://eyewiki.org/Car..."

Main Page

2025-11-17T03:00:10Z

Jack.Dewey: /* Articles in need of Review */

== Welcome to OtoWiki! ==

We are still under construction right now. Please check back soon once we have the main page up and running.

If you are an ENT resident, fellow, or faculty and would like to contribute to this project, please make an account and follow the steps to becoming a verified user. Only verified ENT residents/fellows/staff are allowed to edit pages.

If you have any questions, please reach out to '''OtoWiki.Staff@gmail.com'''.

== How to Get Started ==
=== Making an Account ===
Anyone is allowed to make an account. Follow the link in the top right corner to Log In and select "Create Account". Your username should be FirstName.LastName if you intend to get editing access. Once you have an account you can email me directly at '''OtoWiki.Staff@gmail.com''' to get added to the approved editors list if you are an ENT resident, fellow, or staff. In the future this will also require you to have your name and institution on your account page, but this has not been set up yet.

=== Making and Editing Pages ===
Once you have an account, you are free to edit the pages as you see fit. Please cite all sources where appropriate. If you are adding images to the articles, please ensure they are your own and appropriately deidentified or are open-source images that are appropriately cited.

You'll notice that all of the pages have the same overall architecture - this is intentional to keep the site consistent. You can find the templates for article style below. If a section is in the style guide but not relevant (such as histology or genetics), feel free to remove that section. The infobox template needs to be placed at the top of the article so that it is in the correct position at the top of the page.
* [[Template:Article Disease|Article Template]]
* [[Template:Infobox Disease|Infobox Template]]

== Areas in Need of Improvement ==
The wiki is still in its infancy, and many articles need to be improved upon. As new articles are made and partially completed, please add them to the list below so that others can jump in and add to the article. As this becomes a more established wiki this list will be migrated off of the main page to its own page, and we can work on making the main page a little more fun.

=== Articles in Need of Review ===
These are the articles that have content in every section but could use a grammar review and some bolstering.
* [[Juvenile Nasopharyngeal Angiofibroma]]
* [[Paraganglioma]]

=== Articles with Minor Deficits ===
These are the articles missing a only some of the content in the outline.
* [[Bezold's Abscess|Bezold Abscess]]
* [[Collet Sicard Syndrome]]
* [[Pyriform Aperture Stenosis]]
* [[Villaret Syndrome]]

=== Articles with Major Deficits ===
These are the articles missing a significant portion of the content in the outline.
* [[Acute Invasive Fungal Sinusitis]]
* [[Acute Mastoiditis]]
* [[Cavernous Sinus Thrombosis]]
* [[Citelli Abscess]]
* [[Concha Bullosa]]
* [[Encephalocele]]
* [[Glioma]]
* [[Jackson Syndrome]]
* [[Jugular Foramen Syndrome]]
* [[Killian-Jamieson Diverticulum]]
* [[Labyrinthitis]]
* [[Laimer's Diverticulum]]
* [[Nasal dermoid]]
* [[Orbital Apex Syndrome]]
* [[Schmidt Syndrome]]
* [[Superior Orbital Fissure Syndrome]]
* [[Tapia Syndrome]]
* [[Zenker's Diverticulum]]

Main Page

2025-11-17T02:53:03Z

Jack.Dewey: /* Areas in Need of Improvement */

== Welcome to OtoWiki! ==

We are still under construction right now. Please check back soon once we have the main page up and running.

If you are an ENT resident, fellow, or faculty and would like to contribute to this project, please make an account and follow the steps to becoming a verified user. Only verified ENT residents/fellows/staff are allowed to edit pages.

If you have any questions, please reach out to '''OtoWiki.Staff@gmail.com'''.

== How to Get Started ==
=== Making an Account ===
Anyone is allowed to make an account. Follow the link in the top right corner to Log In and select "Create Account". Your username should be FirstName.LastName if you intend to get editing access. Once you have an account you can email me directly at '''OtoWiki.Staff@gmail.com''' to get added to the approved editors list if you are an ENT resident, fellow, or staff. In the future this will also require you to have your name and institution on your account page, but this has not been set up yet.

=== Making and Editing Pages ===
Once you have an account, you are free to edit the pages as you see fit. Please cite all sources where appropriate. If you are adding images to the articles, please ensure they are your own and appropriately deidentified or are open-source images that are appropriately cited.

You'll notice that all of the pages have the same overall architecture - this is intentional to keep the site consistent. You can find the templates for article style below. If a section is in the style guide but not relevant (such as histology or genetics), feel free to remove that section. The infobox template needs to be placed at the top of the article so that it is in the correct position at the top of the page.
* [[Template:Article Disease|Article Template]]
* [[Template:Infobox Disease|Infobox Template]]

== Areas in Need of Improvement ==
The wiki is still in its infancy, and many articles need to be improved upon. As new articles are made and partially completed, please add them to the list below so that others can jump in and add to the article. As this becomes a more established wiki this list will be migrated off of the main page to its own page, and we can work on making the main page a little more fun.

=== Articles in need of Review ===
These are the articles that have content in every section but could use a grammar review and some bolstering.
* [[Juvenile Nasopharyngeal Angiofibroma]]
* [[Paraganglioma|Paraganglioma]]

=== Articles with Minor Deficits ===
These are the articles missing a only some of the content in the outline.
* [[Bezold's Abscess|Bezold Abscess]]
* [[Collet Sicard Syndrome|Collet Sicard Syndrome]]
* [[Pyriform Aperture Stenosis|Pyriform Aperture Stenosis]]
* [[Villaret Syndrome|Villaret Syndrome]]

=== Articles with Major Deficits ===
These are the articles missing a significant portion of the content in the outline.
* [[Acute Invasive Fungal Sinusitis]]
* [[Acute Mastoiditis]]
* [[Cavernous Sinus Thrombosis]]
* [[Citelli Abscess]]
* [[Concha Bullosa]]
* [[Encephalocele]]
* [[Glioma]]
* [[Jackson Syndrome|Jackson Syndrome]]
* [[Jugular Foramen Syndrome]]
* [[Killian-Jamieson Diverticulum]]
* [[Labyrinthitis]]
* [[Laimer's Diverticulum]]
* [[Nasal dermoid]]
* [[Orbital Apex Syndrome]]
* [[Schmidt Syndrome|Schmidt Syndrome]]
* [[Superior Orbital Fissure Syndrome]]
* [[Tapia Syndrome|Tapia Syndrome]]
* [[Zenker's Diverticulum|Zenker's Diverticulum]]

Juvenile Nasopharyngeal Angiofibroma

2025-08-16T18:46:50Z

Jack.Dewey: /* Surgical Management */

{{infobox Disease
|Title =
|Aliases =
|Image = [[File:Nasopharyngeal angiofibroma - 2 - high mag.jpg]]
|Caption = Histologic section of a JNA
|ICD-9 = 210.7
|ICD-10 = D10.6
|MeSH =
|Gene =
|Locus =
|OMIM =
|EyeWiki =
|Radiopaedia = [https://radiopaedia.org/articles/juvenile-nasopharyngeal-angiofibroma?lang=us Juvenile Nasopharyngeal Angiofibroma]
|Pathology = [https://www.pathologyoutlines.com/topic/nasalangiofibroma.html Nasal Angiofibroma]
}}

== Overview ==
'''Juvenile nasopharyngeal angiofibroma (JNA)''' is a benign vascular neoplasm of the nasal cavity and nasopharynx that classically presents in adolescent boys.

== Pathophysiology ==
=== Relevant Anatomy ===
JNA's typically originate in the region of the sphenopalatine artery (SPA) in the posterior nasal cavity/nasopharynx. From there, they can extend into the paranasal sinuses, pterygopalatine fossa (PPF), infratemporal fossa (ITF), orbit, or cranial vault.

<gallery>
Pterygopalatine fossa.jpg|Cadaveric model of the PPF
Schematic diagram showing the bony anatomy and main neural connections of the PPF.png|Neural contents of the PPF
Gray511.png|Maxillary artery branches, with SPA at the distal end
Gerrish's Text-book of Anatomy (1902) - Fig. 243.png|Location of the sphenopalatine foramen
Basilar process and palatine process.jpg|Location of the ITF
</gallery>

=== Disease Etiology ===
JNA's are fibrovascular tumors that arise from the posterior lateral nasal cavity near the choana. There is substantial debate about the exact circumstances and location that lead to the formation of a JNA, but they are generally accepted to form near the region of the sphenopalatine foramen. Proposed theories regarding their etiology include androgen receptor-related growth,<ref name="Liu 2015">Liu Z, Wang J, Wang H, Wang D, Hu L, Liu Q, Sun X. Hormonal receptors and vascular endothelial growth factor in juvenile nasopharyngeal angiofibroma: immunohistochemical and tissue microarray analysis. Acta oto-laryngologica. 2015 Jan 2;135(1):51-7. https://doi.org/10.3109/00016489.2014.952774</ref> incomplete regression of a branchial artery during embryogenesis,<ref name="Schick 2002">Schick B, Plinkert PK, Prescher A. Aetiology of angiofibromas: reflection on their specific vascular component. Laryngo-rhino-otologie. 2002 Apr 1;81(4):280-4. https://doi.org/10.1055/s-2002-25322</ref> oncogenic mutations in C-MYC and C-KIT,<ref name="Pandey 2017">Pandey P, Mishra A, Tripathi AM, Verma V, Trivedi R, Singh HP, Kumar S, Patel B, Singh V, Pandey S, Pandey A. Current molecular profile of juvenile nasopharyngeal angiofibroma: first comprehensive study from India. The Laryngoscope. 2017 Mar;127(3):E100-6. https://doi.org/10.1002/lary.26250</ref> and a wide variety of other genetic aberrations.

===Epidemiology===
Juvenile nasopharyngeal angiofibroma has been reported to be the most common benign neoplasm of the nasopharynx in young males. It accounts for 0.05% to 0.5% of all head and neck tumors, with a reported incidence of 1 in 5,000 to 1 in 60,000 in the US annually.<ref name="Boghani 2013">Boghani Z, Husain Q, Kanumuri VV, Khan MN, Sangvhi S, Liu JK, Eloy JA. Juvenile nasopharyngeal angiofibroma: a systematic review and comparison of endoscopic, endoscopic‐assisted, and open resection in 1047 cases. The Laryngoscope. 2013 Apr;123(4):859-69. https://doi.org/10.1002/lary.23843</ref><ref name="Ferouz 1995">Ferouz AS, Mohr RM, Paul P. Juvenile nasopharyngeal angiofibroma and familial adenomatous polyposis: an association?. Otolaryngology—Head and Neck Surgery. 1995 Oct;113(4):435-9. https://doi.org/10.1016/S0194-59989570081-1</ref><ref name="Huang 2014">Huang Y, Liu Z, Wang J, Sun X, Yang L, Wang D. Surgical management of juvenile nasopharyngeal angiofibroma: analysis of 162 cases from 1995 to 2012. The Laryngoscope. 2014 Aug;124(8):1942-6. https://doi.org/10.1002/lary.24522</ref> It classically presents in adolescent males, with a typical age of presentation between 13 and 22 years old.<ref name ="Newman 2023">Newman M, Nguyen TB, McHugh T, Reddy K, Sommer DD. Early-onset juvenile nasopharyngeal angiofibroma (JNA): a systematic review. Journal of Otolaryngology-Head & Neck Surgery. 2023 Dec;52(1):s40463-023. https://doi.org/10.1186/s40463-023-00687-w</ref>

=== Genetics ===
There is no identified definitive genetic locus that predisposes patients to JNA's. However, there have been a number of studies looking at the various genetic components that could influence their formation.<ref name="Doody 2019">Doody J, Adil EA, Trenor III CC, Cunningham MJ. The genetic and molecular determinants of juvenile nasopharyngeal angiofibroma: a systematic review. Annals of Otology, Rhinology & Laryngology. 2019 Nov;128(11):1061-72. https://doi.org/10.1177/0003489419850194</ref> Due to fact that JNA's classically form in adolescent males, there have been several studies focused on the possible hormonal influence on their formation and growth. JNA's tend to have an abundance of progesterone and estradiol, but a relatively lower concentration of testosterone and dihydrotestosterone.<ref name="Kumagami 05 1993">Kumagami H. Estradiol, dihydrotestosterone, and testosterone in juvenile nasopharyngeal angiofibroma tissue. American Journal of Rhinology. 1993 May;7(3):101-4. https://doi.org/10.2500/105065893781976393</ref><ref name="Kumagami 01 1993">Kumagami H. Sex hormones in juvenile nasopharyngeal angiofibroma tissue. Auris Nasus Larynx. 1993 Jan 1;20(2):131-5. https://doi.org/10.1016/S0385-8146(12)80240-9</ref> Supplemental testosterone was trialed in JNA patients, but both tumor growth<ref name="Johnsen 1966">Johnsen S, Kloster JH, Schiff M. The action of hormones on juvenile nasopharyngeal angiofibroma. Acta Oto-Laryngologica. 1966 Jan 1;61(1-6):153-60. https://doi.org/10.3109/00016486609127052</ref> and tumor recurrence<ref name="Riggs 2010">Riggs S, Orlandi RR. Juvenile nasopharyngeal angiofibroma recurrence associated with exogenous testosterone therapy. Head & Neck: Journal for the Sciences and Specialties of the Head and Neck. 2010 Jun;32(6):812-5. https://doi.org/10.1002/hed.21152</ref> have been documented. Papers investigating potential targeted therapy markers have looked at steroid hormones, chromosomal abnormalities, growth factors, and other intracellular molecular targets.<ref name="Doody 2019"></ref> There have been studies investigating a possible link between Familial Adenomatous Polyposis (FAP) and JNA's via the adenomatous polyposis coli (APC)/β-catenin pathway, but no specific genetic link has been identified as of yet.<ref name="Guertl 2000">Guertl B, Beham A, Zechner R, Stammberger H, Hoefler G. Nasopharyngeal angiofibroma: an AM-Gene-Associated tumor?. Human pathology. 2000 Nov 1;31(11):1411-3.</ref>

=== Histology ===
Grossly on histologic section there are large fibrous/collagenous sections with fibroblasts, as well as vascular spaces of various sizes throughout the tumor.<ref name="Xu 2020">Xu B. Nasopharyngeal angiofibroma. PathologyOutlines.com website. https://www.pathologyoutlines.com/topic/nasalangiofibroma.html. Accessed November 17th, 2024.</ref> The stromal cells have an abundance of beta-catenin relative to the vascular endothelial cells.

<gallery>
JNA 30x.png|H&E stained section of green inked vascular channel (30x)
Beta Catenin JNA.png|Abnormal nuclear expression in stromal cells (blue arrow), compared with membranous / cytoplasmic staining in the endothelial cells (red arrow)
</gallery>

== Diagnosis ==
=== Patient History ===
Patients may have a history of any of the following symptoms:
* Unilateral nasal obstruction
* Recurrent epistaxis
* High-volume epistaxis
* Eustachian tube dysfunction
* Headache
* Facial swelling
* Anosmia / Hyposmia
* Cranial neuropathy
* Vision changes

=== Physical Examination ===
Many patients will not have obvious findings on anterior rhinoscopy. A large vascular-appearing mass is commonly fond on nasal endoscopy on the posterior lateral nasal sidewall near the region of the sphenopalatine artery. Note that '''''these hypervascular masses should not be biopsied''''', as this may result in significant bleeding that is hard to control in the clinic setting.

=== Laboratory Tests ===
Laboratory tests are not commonly useful in the diagnosis of JNA's, but should be performed preoperatively in the case of a planned surgical resection.
* Hemoglobin / Hematocrit
* Platelet level
* Platelet function (if family history of platelet dysfunction)
* PT / INR
* Blood Type and Screen (all patients)
* Blood Type and Cross (patients with advanced stage tumors where a significant blood loss is expected)

=== Imaging ===
[[File:Holman-Miller Sign.png|right|thumb|300 px|Axial CT scan of a JNA showing a right-sided Holman Miller sign]]
CT scan, angiography, and MRI are all useful in the workup of JNA's and have differing benefits.<ref name="Gaillard 2008">Gaillard F, Walizai T, Bell D, et al. Juvenile nasopharyngeal angiofibroma. Reference article, Radiopaedia.org (Accessed on 17 Nov 2024) https://doi.org/10.53347/rID-1541</ref> CT is typically the first imaging modality ordered in these patients, and is helpful for delineating the extent of bony erosion. Angiography demonstrates the extent of the mass well, and is frequently used in pre-operative embolization 24-48 hours before surgical resection. A majority of JNA's are supplied by the external carotid system, either by the maxillary artery (SPA) or the ascending pharyngeal artery.<ref name="Gaillard 2008"></ref> A lesser fraction of tumors are supplied by the internal carotid system (ophthalmic or sphenoidal branches), and this is typically in larger masses with intracranial invasion. MRI can be used to assess for intraorbital or intracranial extension. JNA's are T1 intermediate, T2 heterogenous with flow voids, and enhance with Gadolinium. The following are general characteristics often found in imaging of JNA's:
* Highly vascular nasopharyngeal mass
* Holman-Miller Sign<ref name="Niknejad 2013">Niknejad M, Tatco V, Bell D, et al. Holman-Miller sign (maxillary sinus). Reference article, Radiopaedia.org (Accessed on 17 Nov 2024) https://doi.org/10.53347/rID-21804</ref>: Anterior displacement of the posterior maxillary wall due to mass effect from the tumor
* Widening of the PMF or PPF
* Erosion of the pterygoid plate, especially the medial plate

There are several classification systems that have been proposed for JNAs based on imaging findings.

{| class="wikitable", style="margin-left: auto; margin-right: auto; border: none; text-align: center"
|+ Classification Systems for Juvenile Nasopharyngeal Angiofibromas
|-
! Classification System !! style=width:15em | Stage I !! style=width:15em | Stage II !! style=width:15em | Stage III !! style=width:15em | Stage IV !! style=width:15em | Stage V
|-
| Andrews (1989)<ref name="Andrews 1989">Andrews JC, Fisch U, Aeppli U, Valavanis A, Makek MS. The surgical management of extensive nasopharyngeal angiofibromas with the infratemporal fossa approach. The Laryngoscope. 1989 Apr;99(4):429-37. https://doi.org/10.1288/00005537-198904000-00013</ref> || Confined to NP || Invading one of the following with evidence of bony erosion: PPF, MS, ES, or SS || Invading ITF or orbit '''IIIa''': No intracranial extension '''IIIb''': Extradural (parasellar) extension || Intradural extension '''IVa''': No infiltration of CS, PF, or OC '''IVb''': Infiltration of CS, PF, or OC || --
|-
| Chandler (1984)<ref name="Chandler 1984">Chandler JR, Moskowitz L, Goulding R, Quencer RM. Nasopharyngeal angiofibromas: staging and management. Annals of Otology, Rhinology & Laryngology. 1984 Jul;93(4):322-9. https://doi.org/10.1177/000348948409300408</ref> || Confined to NP || Extension into the nasal cavity, SS, or both || Extension into any of the following: antrum, ES, PMF, ITF, orbit, or cheek || Intracranial extension || --
|-
| Onerci (2006)<ref name="Onerci 2006">Onerci M, Oğretmenoğlu O, Yücel T. Juvenile nasopharyngeal angiofibroma: a revised staging system. Rhinology. 2006 Mar 1;44(1):39-45. PMID: 16550949</ref>|| Nose, NP, ES, and SS or minimal extension into PMF || MS involvement, full occupation of PMF, extension to anterior cranial fossa, limited extension into ITF || Deep extension into cancellous bone at pterygoid base or body and GW of sphenoid, significant lateral extension into ITF or pterygoid plates, orbital involvement, CS obliteration || Intracranial extension between pituitary gland and ICA, tumor localization lateral to ICA, middle fossa extension and extensive intracranial extension || --
|-
| Radkowski (1996)<ref name="Radkowski 1996">Radkowski D, McGill T, Healy GB, Ohlms L, Jones DT. Angiofibroma: changes in staging and treatment. Archives of Otolaryngology–Head & Neck Surgery. 1996 Feb 1;122(2):122-9. https://doi.org/10.1001/archotol.1996.01890140012004</ref>|| '''IA''': Confined to nose or NP '''IB''': Extends into one or more sinuses || '''IIa''': Minimal extension into medial PMF '''IIb''': Full occupation of PMF with local mass effect '''IIc''': Extension into ITF, cheek, or posterior to pterygoid plates || Erosion of skull base '''IIIa''': Minimal skull base involvement '''IIIb''': Extensive intracranial extension, with or without invasion into CS || -- || --
|-
| Sessions (1981)<ref name="Sessions 1981">Sessions RB, Bryan RN, Naclerio RM, Alford BR. Radiographic staging of juvenile angiofibroma. Head & neck surgery. 1981 Mar;3(4):279-83. https://doi.org/10.1002/hed.2890030404</ref> || '''IA''': Confined to nose or NP '''IB''': Extends into one or more sinuses || '''IIa''': Minimal extension into PMF '''IIb''': Full occupation of PMF with or without orbital erosion '''IIc''': ITF with or without cheek extension || Intracranial extension || -- || --
|-
| UPMC (2010)<ref name="Snyderman 2010">Snyderman CH, Pant H, Carrau RL, Gardner P. A new endoscopic staging system for angiofibromas. Archives of Otolaryngology–Head & Neck Surgery. 2010 Jun 21;136(6):588-94. https://doi.org/10.1001/archoto.2010.83</ref> || Nasal cavity, medial PPF || Paranasal sinuses, lateral PPF; no residual vascularity || Skull base erosion, orbit, ITF involvement; no residual vascularity || Skull base erosion, orbit, ITF involvement; residual vascularity || Intracranial extension with residual vascularity '''M''': Medial extension '''L''': Lateral extension
|-
|+ style="caption-side:bottom; font-weight: normal;"|''Abbreviations: Cavernous sinus (CS), Ethmoid sinus (ES), Infratemporal fossa (ITF), Internal carotid artery (ICA), Maxillary sinus (MS), Nasopharynx (NP), Optic chiasm (OC), Pituitary fossa (PF), Pterygomaxillary fissure (PMF), Pterygopalatine fossa (PPF), Sphenoid sinus (SS)''
|}

=== Differential Diagnosis ===
In the case of a nasopharyngeal mass, the following should also be considered:
* Clival chondroma
* Encephalocele
* Esthesioneuroblastoma
* Inverted papilloma
* Nasal polyp
* Nasopharyngeal carcinoma
* Nasopharyngeal teratoma
* Rhabdomyosarcoma
* Vascular malformation

== Management ==
=== Medical Management ===
There is no approved medical treatment for management of juvenile nasopharyngeal angiofibromas. The treatment is primarily surgical. Medical management focuses on optimizing patients before surgery, such as addressing any coagulopathies or addressing underlying anemia.

=== Surgical Management ===
Historically, surgical management of JNAs involved aggressive open approaches, including transpalatal, lateral rhinotomy, midface degloving, or infratemporal fossa craniotomy approaches.<ref name="Renkonen 2011">Renkonen S, Hagström J, Vuola J, Niemelä M, Porras M, Kivivuori SM, Leivo I, Mäkitie AA. The changing surgical management of juvenile nasopharyngeal angiofibroma. European archives of oto-rhino-laryngology. 2011 Apr;268:599-607. https://doi.org/10.1007/s00405-010-1383-z</ref> In recent years, there has been a transition towards transnasal endoscopic approaches. This has resulted in better visualization during surgery and a subsequent improvement in intraoperative blood loss, need for blood transfusions,<ref name="Oliveira 2012">Oliveira JA, Tavares MG, Aguiar CV, Azevedo JF, Sousa JR, Almeida PC, Gomes EF. Comparison between endoscopic and open surgery in 37 patients with nasopharyngeal angiofibroma. Brazilian Journal of otorhinolaryngology. 2012;78:75-80. https://doi.org/10.1590/S1808-86942012000100012</ref> and recurrence rates.<ref name="Bosraty 2011">Bosraty H, Atef A, Aziz M. Endoscopic vs. open surgery for treating large, locally advanced juvenile angiofibromas: A comparison of local control and morbidity outcomes. ENT: Ear, Nose & Throat Journal. 2011 Nov 1;90(11). PMID: 22109921</ref> Prior to surgery, preoperative embolization should be considered to limit blood loss intraoperatively.

An example of a combined endoscopic transnasal and trans-maxillary (Caldwell-Luc) approach can be seen here:
https://www.youtube.com/watch?v=gYX6NgjAj5Q

== Outcomes ==
=== Complications ===
The primary concerns in the surgical management of juvenile nasopharyngeal angiofibroma are bleeding and recurrence rates. Iatrogenic injuries to structures of the orbit (such as ophthalmoplegia) and skull base (such as facial paresthesias) are also a concern depending on the degree of tumor extension.

==== Bleeding ====
Intraoperative bleeding rates vary based on technique, whether preoperative embolization was used, and tumor staging. Endoscopic surgery is generally accepted to have lower intraoperative blood loss relative to more traditional open approaches.<ref name="Oliveira 2012"></ref> Preoperative embolization has also been found to significantly lower intraoperative blood loss. One study of 47 patients (primarily Radkowski stages I-II) found that patients who underwent preoperative embolization had a mean estimated blood loss (EBL) of 770 mL, and patients who did not undergo embolization had a mean EBL of more than 1,400 mL.<ref name="Ardehali 2010">Ardehali MM, Ardestani SH, Yazdani N, Goodarzi H, Bastaninejad S. Endoscopic approach for excision of juvenile nasopharyngeal angiofibroma: complications and outcomes. American journal of otolaryngology. 2010 Sep 1;31(5):343-9. https://doi.org/10.1016/j.amjoto.2009.04.007</ref> The same study noted that embolized patients had a mean hospital stay of 1.8 days, vs 3.1 days for all patients in the study. A systematic review of advance-stage JNAs with intracranial extension found a mean EBL of 1,700 mL and a similar reduction in EBL following preoperative embolization.<ref name="Leong 2013">Leong SC. A systematic review of surgical outcomes for advanced juvenile nasopharyngeal angiofibroma with intracranial involvement. The Laryngoscope. 2013 May;123(5):1125-31. https://doi.org/10.1002/lary.23760</ref>

==== Recurrence ====
Recurrence rates are similarly related to surgical technique and tumor stage. Endoscopic approaches have been documented to have an improved recurrence rate relative to open approaches.<ref name="Bosraty 2011"></ref> A systematic review of 92 studies totaling 821 patients found a recurrence rate of about 10%, as well as 7.7% with residual tumor.<ref name="Khoueir 2013">Khoueir N, Nicolas N, Rohayem Z, Haddad A, Abou Hamad W. Exclusive endoscopic resection of juvenile nasopharyngeal angiofibroma: a systematic review of the literature. Otolaryngology–Head and Neck Surgery. 2014 Mar;150(3):350-8. https://doi.org/10.1177/0194599813516605</ref> Studies looking at more invasive tumors have a higher rate of recurrence of approximately 18%.<ref name="Leong 2013"></ref>

=== Prognosis ===
Recurrence rates can be very high and increase with increased stage at the time of surgery. Recurrence has been reported from 22%<ref name="Bleier 2009">Bleier BS, Kennedy DW, Palmer JN, Chiu AG, Bloom JD, O'Malley Jr BW. Current management of juvenile nasopharyngeal angiofibroma: a tertiary center experience 1999–2007. American journal of rhinology & allergy. 2009 May;23(3):328-30. https://doi.org/10.2500/ajra.2009.23.3322</ref> to as high as 61% in advanced disease.<ref name="Huang 2014">Huang Y, Liu Z, Wang J, Sun X, Yang L, Wang D. Surgical management of juvenile nasopharyngeal angiofibroma: analysis of 162 cases from 1995 to 2012. The Laryngoscope. 2014 Aug;124(8):1942-6. https://doi.org/10.1002/lary.24522</ref>

== References ==
<references />

Pyriform Aperture Stenosis

2025-07-21T19:11:00Z

Jack.Dewey: /* Disease Etiology */

{{infobox Disease
|Title =
|Aliases = Congenital Nasal Pyriform Aperture Stenosis (CNPAS)
|Image = [[Human skull with piriform aperture circled.png]]
|Caption =
|ICD-9 = 748.0
|ICD-10 = Q30.8
|MeSH =
|Gene =
|Locus =
|OMIM =
|EyeWiki =
|Radiopaedia = [https://radiopaedia.org/articles/pyriform-aperture-stenosis?lang=us Pyriform Aperture Stenosis]
|Pathology =
}}

== Overview ==
'''Pyriform aperture stenosis''' is a congenital malformation resulting in a narrowed pyriform aperture and subsequent nasal obstruction.

=== History ===
The earliest described case of congenital pyriform aperture stenosis repair in the literature was by Douglas in 1952.<ref>DOUGLAS B. The relief of vestibular nasal obstruction by partial resection of the nasal process of the superior maxilla. Plastic and Reconstructive Surgery. 1952 Jan 1;9(1):42-51.</ref>

== Pathophysiology ==
=== Relevant Anatomy ===
Key anatomy includes the structures of the nasal cavity and midface.
<gallery>
Human skull with piriform aperture circled.png | Skull with highlighted pyriform aperture
Slide2hal.JPG | Midface anatomy
</gallery>

=== Disease Etiology ===
Congenital pyriform aperture stenosis is a consequence of failed recanalization of the nasal cavity during development. After the anterior nares are formed from fusion of the lateral and medial nasal processes (at approximately week 8 of gestation), the nasal cavity is blocked by an epithelial plug. After approximately 24 weeks, this epithelial plug is supposed to resorb, resulting in a patent nasal airway. It is though that failure of this process results in a stenotic pyriform aperture at birth.<ref>Papesch E, Papesch M. The nasal pyriform aperture and its importance. Otorhinolaryngol Head Neck Surg. 2016;1(4):89-91.</ref>

=== Genetics ===
There is no consistent genetic variant that has been identified as causative for congenital pyriform aperture stenosis. One study was able to identify genetic variants in two cases: a 2p16.3 deletion and a 7q36 deletion, the latter of which resulted in alterations to the Sonic Hedgehog gene.<ref>Ruda J, Grischkan J, Allarakhia Z. Radiologic, genetic, and endocrine findings in isolated congenital nasal pyriform aperture stenosis patients. International Journal of Pediatric Otorhinolaryngology. 2020 Jan 1;128:109705.</ref>

== Diagnosis ==
=== Patient History ===
It is important to take a focused history related to any airway or feeding symptoms, including:
* Prolonged feeding times
* Needing frequent breaks during feeds
* Cyanosis or increased respiratory effort, especially with feeding
* BRUE's
* Difficulty placing nasogastric tubes
* Family history of genetic conditions with anatomic malformations

=== Physical Examination ===
Physical exam can be nonspecific aside from nasal congestion / narrow nasal cavities. Key findings can include:
* Difficulty passing nasogastric tubes
* Narrow anterior nasal cavity on flexible or rigid nasal endoscopy

Other key features of midline defects should be considered, as this may present as a part of the holoprosencephaly spectrum. With midline defects, there can be endocrine dysfunction as a part of developmental issues related to the hypothalamic-pituitary axis (HPA). As many as 75% of cases may also present with a solitary maxillary central incisor (SMCI).<ref>Lo FS, Lee YJ, Lin SP, Shen EY, Huang JK, Lee KS. Solitary maxillary central incisor and congenital nasal pyriform aperture stenosis. European journal of pediatrics. 1998 Dec;157(1):39-44.</ref>

=== Imaging ===
CT maxillofacial will demonstrate decreased pyriform aperture diameter, especially on axial view. A pyriform aperture width of less than 11 mm is diagnostic for pyriform aperture stenosis.<ref>Belden CJ, Mancuso AA, Schmalfuss IM. CT features of congenital nasal piriform aperture stenosis: initial experience. Radiology. 1999 Nov;213(2):495-501.</ref> MRI should be considered in these patients in order to assess for other midline variants as a part of the holoprosencephaly spectrum.

=== Differential Diagnosis ===
* [[Choanal Atresia|Choanal Atresia]]

== Management ==
=== Medical Management ===
=== Surgical Management ===

== Outcomes ==
=== Complications ===
=== Prognosis ===

== References ==
<references />

Pyriform Aperture Stenosis

2025-07-21T19:04:12Z

Jack.Dewey: /* Physical Examination */

{{infobox Disease
|Title =
|Aliases = Congenital Nasal Pyriform Aperture Stenosis (CNPAS)
|Image = [[Human skull with piriform aperture circled.png]]
|Caption =
|ICD-9 = 748.0
|ICD-10 = Q30.8
|MeSH =
|Gene =
|Locus =
|OMIM =
|EyeWiki =
|Radiopaedia = [https://radiopaedia.org/articles/pyriform-aperture-stenosis?lang=us Pyriform Aperture Stenosis]
|Pathology =
}}

== Overview ==
'''Pyriform aperture stenosis''' is a congenital malformation resulting in a narrowed pyriform aperture and subsequent nasal obstruction.

=== History ===
The earliest described case of congenital pyriform aperture stenosis repair in the literature was by Douglas in 1952.<ref>DOUGLAS B. The relief of vestibular nasal obstruction by partial resection of the nasal process of the superior maxilla. Plastic and Reconstructive Surgery. 1952 Jan 1;9(1):42-51.</ref>

== Pathophysiology ==
=== Relevant Anatomy ===
Key anatomy includes the structures of the nasal cavity and midface.
<gallery>
Human skull with piriform aperture circled.png | Skull with highlighted pyriform aperture
Slide2hal.JPG | Midface anatomy
</gallery>

=== Disease Etiology ===

=== Genetics ===
There is no consistent genetic variant that has been identified as causative for congenital pyriform aperture stenosis. One study was able to identify genetic variants in two cases: a 2p16.3 deletion and a 7q36 deletion, the latter of which resulted in alterations to the Sonic Hedgehog gene.<ref>Ruda J, Grischkan J, Allarakhia Z. Radiologic, genetic, and endocrine findings in isolated congenital nasal pyriform aperture stenosis patients. International Journal of Pediatric Otorhinolaryngology. 2020 Jan 1;128:109705.</ref>

== Diagnosis ==
=== Patient History ===
It is important to take a focused history related to any airway or feeding symptoms, including:
* Prolonged feeding times
* Needing frequent breaks during feeds
* Cyanosis or increased respiratory effort, especially with feeding
* BRUE's
* Difficulty placing nasogastric tubes
* Family history of genetic conditions with anatomic malformations

=== Physical Examination ===
Physical exam can be nonspecific aside from nasal congestion / narrow nasal cavities. Key findings can include:
* Difficulty passing nasogastric tubes
* Narrow anterior nasal cavity on flexible or rigid nasal endoscopy

Other key features of midline defects should be considered, as this may present as a part of the holoprosencephaly spectrum. With midline defects, there can be endocrine dysfunction as a part of developmental issues related to the hypothalamic-pituitary axis (HPA). As many as 75% of cases may also present with a solitary maxillary central incisor (SMCI).<ref>Lo FS, Lee YJ, Lin SP, Shen EY, Huang JK, Lee KS. Solitary maxillary central incisor and congenital nasal pyriform aperture stenosis. European journal of pediatrics. 1998 Dec;157(1):39-44.</ref>

=== Imaging ===
CT maxillofacial will demonstrate decreased pyriform aperture diameter, especially on axial view. A pyriform aperture width of less than 11 mm is diagnostic for pyriform aperture stenosis.<ref>Belden CJ, Mancuso AA, Schmalfuss IM. CT features of congenital nasal piriform aperture stenosis: initial experience. Radiology. 1999 Nov;213(2):495-501.</ref> MRI should be considered in these patients in order to assess for other midline variants as a part of the holoprosencephaly spectrum.

=== Differential Diagnosis ===
* [[Choanal Atresia|Choanal Atresia]]

== Management ==
=== Medical Management ===
=== Surgical Management ===

== Outcomes ==
=== Complications ===
=== Prognosis ===

== References ==
<references />

Pyriform Aperture Stenosis

2025-07-21T19:01:47Z

Jack.Dewey:

{{infobox Disease
|Title =
|Aliases = Congenital Nasal Pyriform Aperture Stenosis (CNPAS)
|Image = [[Human skull with piriform aperture circled.png]]
|Caption =
|ICD-9 = 748.0
|ICD-10 = Q30.8
|MeSH =
|Gene =
|Locus =
|OMIM =
|EyeWiki =
|Radiopaedia = [https://radiopaedia.org/articles/pyriform-aperture-stenosis?lang=us Pyriform Aperture Stenosis]
|Pathology =
}}

== Overview ==
'''Pyriform aperture stenosis''' is a congenital malformation resulting in a narrowed pyriform aperture and subsequent nasal obstruction.

=== History ===
The earliest described case of congenital pyriform aperture stenosis repair in the literature was by Douglas in 1952.<ref>DOUGLAS B. The relief of vestibular nasal obstruction by partial resection of the nasal process of the superior maxilla. Plastic and Reconstructive Surgery. 1952 Jan 1;9(1):42-51.</ref>

== Pathophysiology ==
=== Relevant Anatomy ===
Key anatomy includes the structures of the nasal cavity and midface.
<gallery>
Human skull with piriform aperture circled.png | Skull with highlighted pyriform aperture
Slide2hal.JPG | Midface anatomy
</gallery>

=== Disease Etiology ===

=== Genetics ===
There is no consistent genetic variant that has been identified as causative for congenital pyriform aperture stenosis. One study was able to identify genetic variants in two cases: a 2p16.3 deletion and a 7q36 deletion, the latter of which resulted in alterations to the Sonic Hedgehog gene.<ref>Ruda J, Grischkan J, Allarakhia Z. Radiologic, genetic, and endocrine findings in isolated congenital nasal pyriform aperture stenosis patients. International Journal of Pediatric Otorhinolaryngology. 2020 Jan 1;128:109705.</ref>

== Diagnosis ==
=== Patient History ===
It is important to take a focused history related to any airway or feeding symptoms, including:
* Prolonged feeding times
* Needing frequent breaks during feeds
* Cyanosis or increased respiratory effort, especially with feeding
* BRUE's
* Difficulty placing nasogastric tubes
* Family history of genetic conditions with anatomic malformations

=== Physical Examination ===
Physical exam can be nonspecific aside from nasal congestion / narrow nasal cavities. Key findings can include:
* Difficulty passing nasogastric tubes
* Narrow anterior nasal cavity on flexible or rigid nasal endoscopy

Other key features of midline defects should be considered, as this may present as a part of the holoprosencephaly spectrum. With midline defects, there can be endocrine dysfunction as a part of developmental issues related to the hypothalamic-pituitary axis (HPA). As many as 75% of cases may also present with a solitary median maxillary central incisor (SMMCI).

=== Imaging ===
CT maxillofacial will demonstrate decreased pyriform aperture diameter, especially on axial view. A pyriform aperture width of less than 11 mm is diagnostic for pyriform aperture stenosis.<ref>Belden CJ, Mancuso AA, Schmalfuss IM. CT features of congenital nasal piriform aperture stenosis: initial experience. Radiology. 1999 Nov;213(2):495-501.</ref> MRI should be considered in these patients in order to assess for other midline variants as a part of the holoprosencephaly spectrum.

=== Differential Diagnosis ===
* [[Choanal Atresia|Choanal Atresia]]

== Management ==
=== Medical Management ===
=== Surgical Management ===

== Outcomes ==
=== Complications ===
=== Prognosis ===

== References ==
<references />

Pyriform Aperture Stenosis

2025-07-21T13:24:21Z

Jack.Dewey:

Pyriform Aperture Stenosis

2025-07-21T13:14:44Z

Jack.Dewey:

Pyriform Aperture Stenosis

2025-07-21T02:15:33Z

Jack.Dewey: /* Differential Diagnosis */

{{infobox Disease
|Title =
|Aliases =
|Image = [[Human skull with piriform aperture circled.png]]
|Caption =
|ICD-9 = 748.0
|ICD-10 = Q30.8
|MeSH =
|Gene =
|Locus =
|OMIM =
|EyeWiki =
|Radiopaedia = [https://radiopaedia.org/articles/pyriform-aperture-stenosis?lang=us Pyriform Aperture Stenosis]
|Pathology =
}}

== Overview ==
'''Pyriform aperture stenosis''' is a congenital malformation resulting in a narrowed pyriform aperture and subsequent nasal obstruction.

=== History ===
---

== Pathophysiology ==
=== Relevant Anatomy ===
Key anatomy includes the structures of the nasal cavity and midface.
<gallery>
Human skull with piriform aperture circled.png | Skull with highlighted pyriform aperture
Slide2hal.JPG | Midface anatomy
</gallery>

=== Disease Etiology ===

=== Genetics ===

== Diagnosis ==
=== Patient History ===
It is important to take a focused history related to any airway or feeding symptoms, including:
* Prolonged feeding times
* Needing frequent breaks during feeds
* Cyanosis or increased respiratory effort, especially with feeding
* BRUE's
* Difficulty placing nasogastric tubes
* Family history of genetic conditions with anatomic malformations

=== Physical Examination ===

=== Imaging ===
CT maxillofacial will demonstrate decreased pyriform aperture diameter, especially on axial view. A pyriform aperture width of less than 11 mm is diagnostic for pyriform aperture stenosis.<ref>Belden CJ, Mancuso AA, Schmalfuss IM. CT features of congenital nasal piriform aperture stenosis: initial experience. Radiology. 1999 Nov;213(2):495-501.</ref>

=== Differential Diagnosis ===
* [[Choanal Atresia|Choanal Atresia]]

== Management ==
=== Medical Management ===
=== Surgical Management ===

== Outcomes ==
=== Complications ===
=== Prognosis ===

== References ==
<references />

Main Page

2025-07-21T02:14:40Z

Jack.Dewey: /* Articles with Minor Deficits */

== Welcome to OtoWiki! ==

We are still under construction right now. Please check back soon once we have the main page up and running.

If you are an ENT resident, fellow, or faculty and would like to contribute to this project, please make an account and follow the steps to becoming a verified user. Only verified ENT residents/fellows/staff are allowed to edit pages.

If you have any questions, please reach out to '''OtoWiki.Staff@gmail.com'''.

== How to Get Started ==
=== Making an Account ===
Anyone is allowed to make an account. Follow the link in the top right corner to Log In and select "Create Account". Your username should be FirstName.LastName if you intend to get editing access. Once you have an account you can email me directly at '''OtoWiki.Staff@gmail.com''' to get added to the approved editors list if you are an ENT resident, fellow, or staff. In the future this will also require you to have your name and institution on your account page, but this has not been set up yet.

=== Making and Editing Pages ===
Once you have an account, you are free to edit the pages as you see fit. Please cite all sources where appropriate. If you are adding images to the articles, please ensure they are your own and appropriately deidentified or are open-source images that are appropriately cited.

You'll notice that all of the pages have the same overall architecture - this is intentional to keep the site consistent. You can find the templates for article style below. If a section is in the style guide but not relevant (such as histology or genetics), feel free to remove that section. The infobox template needs to be placed at the top of the article so that it is in the correct position at the top of the page.
* [[Template:Article Disease|Article Template]]
* [[Template:Infobox Disease|Infobox Template]]

== Areas in Need of Improvement ==
The wiki is still in its infancy, and many articles need to be improved upon. As new articles are made and partially completed, please add them to the list below so that others can jump in and add to the article. As this becomes a more established wiki this list will be migrated off of the main page to its own page, and we can work on making the main page a little more fun.

=== Articles with Major Deficits ===
These are the articles missing a significant portion of the content in the outline.
* [[Acute Invasive Fungal Sinusitis]]
* [[Acute Mastoiditis]]
* [[Cavernous Sinus Thrombosis]]
* [[Citelli Abscess]]
* [[Concha Bullosa]]
* [[Encephalocele]]
* [[Glioma]]
* [[Jackson Syndrome|Jackson Syndrome]]
* [[Jugular Foramen Syndrome]]
* [[Killian-Jamieson Diverticulum]]
* [[Labyrinthitis]]
* [[Laimer's Diverticulum]]
* [[Nasal dermoid]]
* [[Orbital Apex Syndrome]]
* [[Schmidt Syndrome|Schmidt Syndrome]]
* [[Superior Orbital Fissure Syndrome]]
* [[Tapia Syndrome|Tapia Syndrome]]
* [[Zenker's Diverticulum|Zenker's Diverticulum]]

=== Articles with Minor Deficits ===
These are the articles missing a only some of the content in the outline.
* [[Bezold's Abscess|Bezold Abscess]]
* [[Collet Sicard Syndrome|Collet Sicard Syndrome]]
* [[Pyriform Aperture Stenosis|Pyriform Aperture Stenosis]]
* [[Villaret Syndrome|Villaret Syndrome]]

=== Articles in need of Review ===
These are the articles that have content in every section but could use a grammar review and some bolstering.
* [[Juvenile Nasopharyngeal Angiofibroma]]
* [[Paraganglioma|Paraganglioma]]

Pyriform Aperture Stenosis

2025-07-21T02:13:48Z

Jack.Dewey: Created page with "{{infobox Disease |Title = |Aliases = |Image = Human skull with piriform aperture circled.png |Caption = |ICD-9 = 748.0 |ICD-10 = Q30.8 |MeSH = |Gene = |Locus = |OMIM = |EyeWiki = |Radiopaedia = [https://radiopaedia.org/articles/pyriform-aperture-stenosis?lang=us Pyriform Aperture Stenosis] |Pathology = }}..."

{{infobox Disease
|Title =
|Aliases =
|Image = [[Human skull with piriform aperture circled.png]]
|Caption =
|ICD-9 = 748.0
|ICD-10 = Q30.8
|MeSH =
|Gene =
|Locus =
|OMIM =
|EyeWiki =
|Radiopaedia = [https://radiopaedia.org/articles/pyriform-aperture-stenosis?lang=us Pyriform Aperture Stenosis]
|Pathology =
}}

== Overview ==
'''Pyriform aperture stenosis''' is a congenital malformation resulting in a narrowed pyriform aperture and subsequent nasal obstruction.

=== History ===
---

== Pathophysiology ==
=== Relevant Anatomy ===
Key anatomy includes the structures of the nasal cavity and midface.
<gallery>
Human skull with piriform aperture circled.png | Skull with highlighted pyriform aperture
Slide2hal.JPG | Midface anatomy
</gallery>

=== Disease Etiology ===

=== Genetics ===

== Diagnosis ==
=== Patient History ===
It is important to take a focused history related to any airway or feeding symptoms, including:
* Prolonged feeding times
* Needing frequent breaks during feeds
* Cyanosis or increased respiratory effort, especially with feeding
* BRUE's
* Difficulty placing nasogastric tubes
* Family history of genetic conditions with anatomic malformations

=== Physical Examination ===

=== Imaging ===
CT maxillofacial will demonstrate decreased pyriform aperture diameter, especially on axial view. A pyriform aperture width of less than 11 mm is diagnostic for pyriform aperture stenosis.<ref>Belden CJ, Mancuso AA, Schmalfuss IM. CT features of congenital nasal piriform aperture stenosis: initial experience. Radiology. 1999 Nov;213(2):495-501.</ref>

=== Differential Diagnosis ===
* Choanal Atresia

== Management ==
=== Medical Management ===
=== Surgical Management ===

== Outcomes ==
=== Complications ===
=== Prognosis ===

== References ==
<references />

Nasal dermoid

2025-06-25T18:06:13Z

Jack.Dewey:

{{infobox Disease
|Title =
|Aliases =
|Image =
|Caption =
|ICD-9 = 748.1
|ICD-10 = Q30.8
|MeSH =
|Gene =
|Locus =
|OMIM =
|EyeWiki =
|Radiopaedia = [https://radiopaedia.org/articles/nasal-dermoid-cyst?lang=us Nasal Dermoid]
|Pathology = [https://www.pathologyoutlines.com/topic/skintumornonmelanocyticdermoidcyst.html Dermoid Cysts]
}}

== Overview ==
'''Nasal dermoids''' are rare congenital midline nasal masses that form as a result of aberrant migration of mesodermal and ectodermal cells during formation of nasal structures.

=== History ===

== Pathophysiology ==
=== Relevant Anatomy ===
=== Disease Etiology ===
=== Genetics ===
=== Histology ===
=== Epidemiology ===
Nasal dermoids make up approximately 10% of head and neck dermoids, and approximately 1% of all dermoids.<ref>McCaffrey TV, McDonald TJ, Gorenstein A. Dermoid cysts of the nose: review of 21 cases. Otolaryngology–Head and Neck Surgery. 1979 Jan;87(1):52-9.</ref>

== Diagnosis ==
=== Patient History ===
Nasal dermoids are congenital lesions, and should be identifiable from birth. Small dermoids may go unnoticed for some time. Important considerations include any other history of congenital malformations, known genetic syndromes, and history of nasal obstruction.

=== Physical Examination ===
Many dermoids have a sinus tract exiting the skin near the nasal dorsum in the midline. Dimples and fistula tracts may appear as high as the glabella or as inferior as the columella. In the presence of active infection, the underlying cyst cavity may be erythematous, tender to touch, or present with purulent drainage from a patent fistula tract. A widened nasal dorsum or hypertelorism may be present in large dermoids.

=== Laboratory Tests ===
=== Imaging ===
=== Differential Diagnosis ===
Other midline congenital nasal masses should be considered:
* [[Glioma]]
* [[Encephalocele]]

== Management ==
=== Medical Management ===
=== Surgical Management ===

== Outcomes ==
=== Complications ===
=== Prognosis ===

== References ==
<references />

Nasal dermoid

2025-06-25T14:31:11Z

Jack.Dewey: /* Physical Examination */

{{infobox Disease
|Title =
|Aliases =
|Image =
|Caption =
|ICD-9 =
|ICD-10 =
|MeSH =
|Gene =
|Locus =
|OMIM =
|EyeWiki =
|Radiopaedia =
|Pathology =
}}

== Overview ==
'''Nasal dermoids''' are rare congenital midline nasal masses that form as a result of aberrant migration of mesodermal and ectodermal cells during formation of nasal structures.

=== History ===

== Pathophysiology ==
=== Relevant Anatomy ===
=== Disease Etiology ===
=== Genetics ===
=== Histology ===
=== Epidemiology ===
Nasal dermoids make up approximately 10% of head and neck dermoids, and approximately 1% of all dermoids.<ref>McCaffrey TV, McDonald TJ, Gorenstein A. Dermoid cysts of the nose: review of 21 cases. Otolaryngology–Head and Neck Surgery. 1979 Jan;87(1):52-9.</ref>

== Diagnosis ==
=== Patient History ===
Nasal dermoids are congenital lesions, and should be identifiable from birth. Small dermoids may go unnoticed for some time. Important considerations include any other history of congenital malformations, known genetic syndromes, and history of nasal obstruction.

=== Physical Examination ===
Many dermoids have a sinus tract exiting the skin near the nasal dorsum in the midline. Dimples and fistula tracts may appear as high as the glabella or as inferior as the columella. In the presence of active infection, the underlying cyst cavity may be erythematous, tender to touch, or present with purulent drainage from a patent fistula tract. A widened nasal dorsum or hypertelorism may be present in large dermoids.

=== Laboratory Tests ===
=== Imaging ===
=== Differential Diagnosis ===
Other midline congenital nasal masses should be considered:
* [[Glioma]]
* [[Encephalocele]]

== Management ==
=== Medical Management ===
=== Surgical Management ===

== Outcomes ==
=== Complications ===
=== Prognosis ===

== References ==
<references />

Nasal dermoid

2025-06-25T14:26:05Z

Jack.Dewey: /* Patient History */

{{infobox Disease
|Title =
|Aliases =
|Image =
|Caption =
|ICD-9 =
|ICD-10 =
|MeSH =
|Gene =
|Locus =
|OMIM =
|EyeWiki =
|Radiopaedia =
|Pathology =
}}

== Overview ==
'''Nasal dermoids''' are rare congenital midline nasal masses that form as a result of aberrant migration of mesodermal and ectodermal cells during formation of nasal structures.

=== History ===

== Pathophysiology ==
=== Relevant Anatomy ===
=== Disease Etiology ===
=== Genetics ===
=== Histology ===
=== Epidemiology ===
Nasal dermoids make up approximately 10% of head and neck dermoids, and approximately 1% of all dermoids.<ref>McCaffrey TV, McDonald TJ, Gorenstein A. Dermoid cysts of the nose: review of 21 cases. Otolaryngology–Head and Neck Surgery. 1979 Jan;87(1):52-9.</ref>

== Diagnosis ==
=== Patient History ===
Nasal dermoids are congenital lesions, and should be identifiable from birth. Small dermoids may go unnoticed for some time. Important considerations include any other history of congenital malformations, known genetic syndromes, and history of nasal obstruction.

=== Physical Examination ===
=== Laboratory Tests ===
=== Imaging ===
=== Differential Diagnosis ===
Other midline congenital nasal masses should be considered:
* [[Glioma]]
* [[Encephalocele]]

== Management ==
=== Medical Management ===
=== Surgical Management ===

== Outcomes ==
=== Complications ===
=== Prognosis ===

== References ==
<references />

Nasal dermoid

2025-06-25T14:24:36Z

Jack.Dewey:

Nasal dermoid

2025-06-25T14:14:55Z

Jack.Dewey: /* Overview */

Main Page

2025-06-25T13:55:36Z

Jack.Dewey:

== Welcome to OtoWiki! ==

We are still under construction right now. Please check back soon once we have the main page up and running.

If you are an ENT resident, fellow, or faculty and would like to contribute to this project, please make an account and follow the steps to becoming a verified user. Only verified ENT residents/fellows/staff are allowed to edit pages.

If you have any questions, please reach out to '''OtoWiki.Staff@gmail.com'''.

== How to Get Started ==
=== Making an Account ===
Anyone is allowed to make an account. Follow the link in the top right corner to Log In and select "Create Account". Your username should be FirstName.LastName if you intend to get editing access. Once you have an account you can email me directly at '''OtoWiki.Staff@gmail.com''' to get added to the approved editors list if you are an ENT resident, fellow, or staff. In the future this will also require you to have your name and institution on your account page, but this has not been set up yet.

=== Making and Editing Pages ===
Once you have an account, you are free to edit the pages as you see fit. Please cite all sources where appropriate. If you are adding images to the articles, please ensure they are your own and appropriately deidentified or are open-source images that are appropriately cited.

You'll notice that all of the pages have the same overall architecture - this is intentional to keep the site consistent. You can find the templates for article style below. If a section is in the style guide but not relevant (such as histology or genetics), feel free to remove that section. The infobox template needs to be placed at the top of the article so that it is in the correct position at the top of the page.
* [[Template:Article Disease|Article Template]]
* [[Template:Infobox Disease|Infobox Template]]

== Areas in Need of Improvement ==
The wiki is still in its infancy, and many articles need to be improved upon. As new articles are made and partially completed, please add them to the list below so that others can jump in and add to the article. As this becomes a more established wiki this list will be migrated off of the main page to its own page, and we can work on making the main page a little more fun.

=== Articles with Major Deficits ===
These are the articles missing a significant portion of the content in the outline.
* [[Acute Invasive Fungal Sinusitis]]
* [[Acute Mastoiditis]]
* [[Cavernous Sinus Thrombosis]]
* [[Citelli Abscess]]
* [[Concha Bullosa]]
* [[Encephalocele]]
* [[Glioma]]
* [[Jackson Syndrome|Jackson Syndrome]]
* [[Jugular Foramen Syndrome]]
* [[Killian-Jamieson Diverticulum]]
* [[Labyrinthitis]]
* [[Laimer's Diverticulum]]
* [[Nasal dermoid]]
* [[Orbital Apex Syndrome]]
* [[Schmidt Syndrome|Schmidt Syndrome]]
* [[Superior Orbital Fissure Syndrome]]
* [[Tapia Syndrome|Tapia Syndrome]]
* [[Zenker's Diverticulum|Zenker's Diverticulum]]

=== Articles with Minor Deficits ===
These are the articles missing a only some of the content in the outline.
* [[Bezold's Abscess|Bezold Abscess]]
* [[Collet Sicard Syndrome|Collet Sicard Syndrome]]
* [[Villaret Syndrome|Villaret Syndrome]]

=== Articles in need of Review ===
These are the articles that have content in every section but could use a grammar review and some bolstering.
* [[Juvenile Nasopharyngeal Angiofibroma]]
* [[Paraganglioma|Paraganglioma]]

Encephalocele

2025-06-25T13:54:34Z

Jack.Dewey: Created page with "{{infobox Disease |Title = |Aliases = |Image = |Caption = |ICD-9 = |ICD-10 = |MeSH = |Gene = |Locus = |OMIM = |EyeWiki = |Radiopaedia = |Pathology = }} == Overview == === History === == Pathophysiology == === Relevant Anatomy === === Disease Etiology === === Genetics === === Histology === == Diagnosis =..."

{{infobox Disease
|Title =
|Aliases =
|Image =
|Caption =
|ICD-9 =
|ICD-10 =
|MeSH =
|Gene =
|Locus =
|OMIM =
|EyeWiki =
|Radiopaedia =
|Pathology =
}}

== Overview ==
=== History ===

== Pathophysiology ==
=== Relevant Anatomy ===
=== Disease Etiology ===
=== Genetics ===
=== Histology ===

== Diagnosis ==
=== Patient History ===
=== Physical Examination ===
=== Laboratory Tests ===
=== Imaging ===
=== Differential Diagnosis ===
Other midline congenital nasal masses should be considered:
* [[Nasal dermoid]]
* [[Glioma]]

== Management ==
=== Medical Management ===
=== Surgical Management ===

== Outcomes ==
=== Complications ===
=== Prognosis ===

== References ==
<references />

Glioma

2025-06-25T13:54:10Z

{{infobox Disease
|Title =
|Aliases =
|Image =
|Caption =
|ICD-9 =
|ICD-10 =
|MeSH =
|Gene =
|Locus =
|OMIM =
|EyeWiki =
|Radiopaedia =
|Pathology =
}}

== Overview ==
=== History ===

== Pathophysiology ==
=== Relevant Anatomy ===
=== Disease Etiology ===
=== Genetics ===
=== Histology ===

== Diagnosis ==
=== Patient History ===
=== Physical Examination ===
=== Laboratory Tests ===
=== Imaging ===
=== Differential Diagnosis ===
Other midline congenital nasal masses should be considered:
* [[Nasal dermoid]]
* [[Encephalocele]]

== Management ==
=== Medical Management ===
=== Surgical Management ===

== Outcomes ==
=== Complications ===
=== Prognosis ===

== References ==
<references />

Nasal dermoid

2025-06-25T13:53:52Z

{{infobox Disease
|Title =
|Aliases =
|Image =
|Caption =
|ICD-9 =
|ICD-10 =
|MeSH =
|Gene =
|Locus =
|OMIM =
|EyeWiki =
|Radiopaedia =
|Pathology =
}}

== Overview ==
=== History ===

== Pathophysiology ==
=== Relevant Anatomy ===
=== Disease Etiology ===
=== Genetics ===
=== Histology ===

== Diagnosis ==
=== Patient History ===
=== Physical Examination ===
=== Laboratory Tests ===
=== Imaging ===
=== Differential Diagnosis ===
Other midline congenital nasal masses should be considered:
* [[Glioma]]
* [[Encephalocele]]

== Management ==
=== Medical Management ===
=== Surgical Management ===

== Outcomes ==
=== Complications ===
=== Prognosis ===

== References ==
<references />

OtoWiki:About

2024-12-02T19:47:24Z

Jack.Dewey: /* Can I make an account? */

== About OtoWiki ==

OtoWiki is a free site intended to be a reference for anyone interested in the field of otolaryngology, with a focus on providing information for residents, fellows, and staff providing care to patients with ENT concerns. The information presented here is crowdsourced by those in the field of otolaryngology who are interested in collecting and sharing their knowledge on various topics of the head and neck.

== Frequently Asked Questions ==

=== Can I make an account? ===

Yes, anyone can make an account on OtoWiki. To make an account, click the "Create Account" button on the top right corner of any page. Your username should be FirstName.LastName if you intend to get editing access. Otherwise, feel free to make your username anything you like. With an account you can track changes on pages that interest you.

=== How do I get editing access? ===

As of now, only otolaryngology residents, fellows, or staff members are given permission to edit pages. This is intended to minimize page inaccuracies and vandalism. Please ensure that your username is FirstName.LastName. Once you have created the account please email us directly at '''OtoWiki.Staff@gmail.com''' to get added to the list of approved editors.

=== How do I make new pages? ===

Please refer to the [[Help:Getting_Started|Getting Started]] page to learn more about page creation and editing. This can be found at any time on the left sidebar.

OtoWiki:About

2024-12-02T19:34:47Z

Jack.Dewey: /* How do I make new pages? */

== About OtoWiki ==

OtoWiki is a free site intended to be a reference for anyone interested in the field of otolaryngology, with a focus on providing information for residents, fellows, and staff providing care to patients with ENT concerns. The information presented here is crowdsourced by those in the field of otolaryngology who are interested in collecting and sharing their knowledge on various topics of the head and neck.

== Frequently Asked Questions ==

=== Can I make an account? ===

Yes, anyone can make an account on OtoWiki. To make an account, click the "Log in" button on the top right corner of any page. From there, select "Create Account". Your username should be FirstName.LastName if you intend to get editing access. Otherwise, feel free to make your username anything you like. With an account you can track changes on pages that interest you.

=== How do I get editing access? ===

As of now, only otolaryngology residents, fellows, or staff members are given permission to edit pages. This is intended to minimize page inaccuracies and vandalism. Please ensure that your username is FirstName.LastName. Once you have created the account please email us directly at '''OtoWiki.Staff@gmail.com''' to get added to the list of approved editors.

=== How do I make new pages? ===

Please refer to the [[Help:Getting_Started|Getting Started]] page to learn more about page creation and editing. This can be found at any time on the left sidebar.

Help:Creating an Account

2024-12-02T19:33:10Z

Jack.Dewey: Jack.Dewey moved page Help:Creating an Account to Help:Getting Started: Changing this to be more inclusive than just account creation. Will be including page creation and editing tips.

#REDIRECT [[Help:Getting Started]]

Help:Getting Started

2024-12-02T19:33:10Z

Jack.Dewey: Jack.Dewey moved page Help:Creating an Account to Help:Getting Started: Changing this to be more inclusive than just account creation. Will be including page creation and editing tips.

== Creating an account on OtoWiki ==
[Needs Added]

== Verifying your account ==
[Needs Added]

== Editing pages ==
[Needs Added]

OtoWiki:About

2024-12-02T19:30:00Z

Jack.Dewey: /* Frequently Asked Questions */

== About OtoWiki ==

OtoWiki is a free site intended to be a reference for anyone interested in the field of otolaryngology, with a focus on providing information for residents, fellows, and staff providing care to patients with ENT concerns. The information presented here is crowdsourced by those in the field of otolaryngology who are interested in collecting and sharing their knowledge on various topics of the head and neck.

== Frequently Asked Questions ==

=== Can I make an account? ===

Yes, anyone can make an account on OtoWiki. To make an account, click the "Log in" button on the top right corner of any page. From there, select "Create Account". Your username should be FirstName.LastName if you intend to get editing access. Otherwise, feel free to make your username anything you like. With an account you can track changes on pages that interest you.

=== How do I get editing access? ===

As of now, only otolaryngology residents, fellows, or staff members are given permission to edit pages. This is intended to minimize page inaccuracies and vandalism. Please ensure that your username is FirstName.LastName. Once you have created the account please email us directly at '''OtoWiki.Staff@gmail.com''' to get added to the list of approved editors.

=== How do I make new pages? ===

Please refer to the Getting Started*** page to learn more about page creation and editing. This can be found at any time on the left sidebar.

OtoWiki:About

2024-12-02T19:26:31Z

Jack.Dewey: /* Can I make an account? */

OtoWiki:About

2024-12-02T19:24:31Z

Jack.Dewey: Created page with "== About OtoWiki == OtoWiki is a free site intended to be a reference for anyone interested in the field of otolaryngology, with a focus on providing information for residents, fellows, and staff providing care to patients with ENT concerns. The information presented here is crowdsourced by those in the field of otolaryngology who are interested in collecting and sharing their knowledge on various topics of the head and neck. == Frequently Asked Questions == === Can I..."

OtoWiki:General disclaimer

2024-12-02T19:14:28Z

Jack.Dewey: Created page with "== General Disclaimer == The content on OtoWiki is not reviewed by medical professionals for use in the clinical setting before being publicly available. The information provided on the website is strictly for educational or referential purposes. Follow up on cited references and investigate the primary literature before applying information here to clinical practice. By using this site you understand that the content is generated by a group of people that is not affili..."

== General Disclaimer ==

The content on OtoWiki is not reviewed by medical professionals for use in the clinical setting before being publicly available. The information provided on the website is strictly for educational or referential purposes. Follow up on cited references and investigate the primary literature before applying information here to clinical practice. By using this site you understand that the content is generated by a group of people that is not affiliated with OtoWiki. Those that we select to have edit access for articles are intended to be otolaryngology residents, fellows, and staff but we cannot guarantee the veracity of the content they provide.

'''For non-providers''': OtoWiki is not an adequate replacement for a trained medical provider. Do not use OtoWiki as a source of medical advice. If you have questions about the content seen here, please discuss them with your doctor before changing any medications or practices.

OtoWiki:Privacy policy

2024-12-01T18:13:59Z

Jack.Dewey: /* Policies */

== General Statement ==

OtoWiki (referred to as the "website" or "site") is dedicated to protecting the privacy of it's users. The following lists our practices regarding user privacy and the website's data retention policies

== Policies ==

# We only use data collected on this website for purposes related to the website. No information is gathered, requested, stored, or otherwise repurposed for the sake of other ventures or sale to third parties.
# We use technology available to us through MediaWiki packages to protect user data.
# Users are allowed to delete their account at any time. Any edits made while their account was active will still be visible to other users, and their username may still be attached to those edits.
# Content on this website is not necessarily endorsed as accurate by OtoWiki personnel. This wiki is editable by anyone who has been verified to be an otolaryngology resident, fellow, or staff member. As such there may be conflicting information within or between pages, typographical errors, or information that is influenced by personal biases. We will do our best to correct any errors as they become known to us, and we encourage other users with editing privileges to do the same.
# '''For providers''': The information provided on the website is strictly for educational or referential purposes. Follow up on cited references and investigate the primary literature before applying information here to clinical practice.
# '''For other readers''': OtoWiki is not an adequate replacement for a trained medical provider. Do not use OtoWiki as a source of medical advice. If you have questions about the content seen here, please discuss them with your doctor before changing any medications or practices.

If you have any questions about the site's privacy policy that are not addressed by the above section, or have suggestions about how we can further clarify our site's privacy policy, please reach out to our team at '''OtoWiki.Staff@gmail.com'''. Thank you.

OtoWiki:Privacy policy

2024-12-01T18:13:36Z

Jack.Dewey: Created page with "== General Statement == OtoWiki (referred to as the "website" or "site") is dedicated to protecting the privacy of it's users. The following lists our practices regarding user privacy and the website's data retention policies == Policies == # We only use data collected on this website for purposes related to the website. No information is gathered, requested, stored, or otherwise repurposed for the sake of other ventures or sale to third parties. # We use technology a..."

Acute Invasive Fungal Sinusitis

2024-12-01T16:14:02Z

Jack.Dewey:

{{infobox Disease
|Title =
|Aliases = Acute fulminant invasive fungal sinusitis (AFIFS)
|Image = [Mucor Orbital Invasion.jpg]
|Caption = Coronal CT scan demonstrating AIFS with extension into the right orbit
|ICD-9 =
|ICD-10 =
|MeSH =
|Gene =
|Locus =
|OMIM =
|EyeWiki = [https://eyewiki.org/Invasive_Fungal_Infections_of_the_Orbit_and_Sinuses Invasive Fungal Infections]
|Radiopaedia = [https://radiopaedia.org/articles/acute-invasive-fungal-sinusitis?lang=us Acute Invasive Fungal Sinusitis]
}}

== Overview ==
=== History ===

== Pathophysiology ==
=== Relevant Anatomy ===

=== Disease Etiology ===
There are many different fungal species that can be invasive, but the most common are the Zygomycetes (''Muror'', ''Rhizopus'', and ''Rhizomucor'') and ''Aspergillus'' species.

=== Histology ===
Histologic evaluation is necessary to determine the species of invasion, which can influence treatment decisions with respect to antifungal coverage. ''Mucor'' and ''Rhizopus'' are both characterized by non-septate fungal hyphae with 90-degree angle branching. Aspergillus also has non-septate hyphae, but typically has 45-degree angle branching.

<gallery>
Zygomycosis, mucormycosis 1.jpg|Mucormycosis
Zygomycosis, mucormycosis 2.jpg|Mucormycosis
Zygomycosis Mucormycosis (13430751363).jpg|Mucormycosis with intravascular invasion
Aspergillosis, angioinvasive, intravascular (5390967599).jpg|Aspergillosis with intravascular invasion
Aspergillosis, angioinvasive, - GMS stain (5390967417).jpg|Aspergillosis with intravascular invasion, GMS stain
</gallery>

== Diagnosis ==
=== Patient History ===
=== Physical Examination ===
=== Laboratory Tests ===
=== Imaging ===
=== Differential Diagnosis ===

== Management ==
=== Medical Management ===
=== Surgical Management ===

== Outcomes ==
=== Complications ===
=== Prognosis ===

== References ==
<references />

File:Mucor Orbital Invasion.jpg

2024-12-01T16:12:07Z

Jack.Dewey: Coronal CT scan demonstrating acute invasive fungal sinusitis with extension into the right orbit. Courtesy of Radiopaedia. Abdrabou A, Invasive fungal sinusitis. Case study, Radiopaedia.org (Accessed on 01 Dec 2024) https://doi.org/10.53347/rID-28818

== Summary ==
Coronal CT scan demonstrating acute invasive fungal sinusitis with extension into the right orbit. Courtesy of Radiopaedia. Abdrabou A, Invasive fungal sinusitis. Case study, Radiopaedia.org (Accessed on 01 Dec 2024) https://doi.org/10.53347/rID-28818

Main Page

2024-11-18T21:11:08Z

Jack.Dewey:

== Welcome to OtoWiki! ==

We are still under construction right now. Please check back soon once we have the main page up and running.

If you are an ENT resident, fellow, or faculty and would like to contribute to this project, please make an account and follow the steps to becoming a verified user. Only verified ENT residents/fellows/staff are allowed to edit pages.

If you have any questions, please reach out to '''OtoWiki.Staff@gmail.com'''.

== How to Get Started ==
=== Making an Account ===
Anyone is allowed to make an account. Follow the link in the top right corner to Log In and select "Create Account". Your username should be FirstName.LastName if you intend to get editing access. Once you have an account you can email me directly at '''OtoWiki.Staff@gmail.com''' to get added to the approved editors list if you are an ENT resident, fellow, or staff. In the future this will also require you to have your name and institution on your account page, but this has not been set up yet.

=== Making and Editing Pages ===
Once you have an account, you are free to edit the pages as you see fit. Please cite all sources where appropriate. If you are adding images to the articles, please ensure they are your own and appropriately deidentified or are open-source images that are appropriately cited.

You'll notice that all of the pages have the same overall architecture - this is intentional to keep the site consistent. You can find the templates for article style below. If a section is in the style guide but not relevant (such as histology or genetics), feel free to remove that section. The infobox template needs to be placed at the top of the article so that it is in the correct position at the top of the page.
* [[Template:Article Disease|Article Template]]
* [[Template:Infobox Disease|Infobox Template]]

== Areas in Need of Improvement ==
The wiki is still in its infancy, and many articles need to be improved upon. As new articles are made and partially completed, please add them to the list below so that others can jump in and add to the article. As this becomes a more established wiki this list will be migrated off of the main page to its own page, and we can work on making the main page a little more fun.

=== Articles with Major Deficits ===
These are the articles missing a significant portion of the content in the outline.
* [[Acute Invasive Fungal Sinusitis]]
* [[Acute Mastoiditis]]
* [[Cavernous Sinus Thrombosis]]
* [[Citelli Abscess]]
* [[Concha Bullosa]]
* [[Jackson Syndrome|Jackson Syndrome]]
* [[Jugular Foramen Syndrome]]
* [[Killian-Jamieson Diverticulum]]
* [[Labyrinthitis]]
* [[Laimer's Diverticulum]]
* [[Orbital Apex Syndrome]]
* [[Schmidt Syndrome|Schmidt Syndrome]]
* [[Superior Orbital Fissure Syndrome]]
* [[Tapia Syndrome|Tapia Syndrome]]
* [[Zenker's Diverticulum|Zenker's Diverticulum]]

=== Articles with Minor Deficits ===
These are the articles missing a only some of the content in the outline.
* [[Bezold's Abscess|Bezold Abscess]]
* [[Collet Sicard Syndrome|Collet Sicard Syndrome]]
* [[Villaret Syndrome|Villaret Syndrome]]

=== Articles in need of Review ===
These are the articles that have content in every section but could use a grammar review and some bolstering.
* [[Juvenile Nasopharyngeal Angiofibroma]]
* [[Paraganglioma|Paraganglioma]]

Juvenile Nasopharyngeal Angiofibroma

2024-11-18T21:04:22Z

Jack.Dewey:

{{infobox Disease
|Title =
|Aliases =
|Image = [[File:Nasopharyngeal angiofibroma - 2 - high mag.jpg]]
|Caption = Histologic section of a JNA
|ICD-9 = 210.7
|ICD-10 = D10.6
|MeSH =
|Gene =
|Locus =
|OMIM =
|EyeWiki =
|Radiopaedia = [https://radiopaedia.org/articles/juvenile-nasopharyngeal-angiofibroma?lang=us Juvenile Nasopharyngeal Angiofibroma]
|Pathology = [https://www.pathologyoutlines.com/topic/nasalangiofibroma.html Nasal Angiofibroma]
}}

== Overview ==
'''Juvenile nasopharyngeal angiofibroma (JNA)''' is a benign vascular neoplasm of the nasal cavity and nasopharynx that classically presents in adolescent boys.

== Pathophysiology ==
=== Relevant Anatomy ===
JNA's typically originate in the region of the sphenopalatine artery (SPA) in the posterior nasal cavity/nasopharynx. From there, they can extend into the paranasal sinuses, pterygopalatine fossa (PPF), infratemporal fossa (ITF), orbit, or cranial vault.

<gallery>
Pterygopalatine fossa.jpg|Cadaveric model of the PPF
Schematic diagram showing the bony anatomy and main neural connections of the PPF.png|Neural contents of the PPF
Gray511.png|Maxillary artery branches, with SPA at the distal end
Gerrish's Text-book of Anatomy (1902) - Fig. 243.png|Location of the sphenopalatine foramen
Basilar process and palatine process.jpg|Location of the ITF
</gallery>

=== Disease Etiology ===
JNA's are fibrovascular tumors that arise from the posterior lateral nasal cavity near the choana. There is substantial debate about the exact circumstances and location that lead to the formation of a JNA, but they are generally accepted to form near the region of the sphenopalatine foramen. Proposed theories regarding their etiology include androgen receptor-related growth,<ref name="Liu 2015">Liu Z, Wang J, Wang H, Wang D, Hu L, Liu Q, Sun X. Hormonal receptors and vascular endothelial growth factor in juvenile nasopharyngeal angiofibroma: immunohistochemical and tissue microarray analysis. Acta oto-laryngologica. 2015 Jan 2;135(1):51-7. https://doi.org/10.3109/00016489.2014.952774</ref> incomplete regression of a branchial artery during embryogenesis,<ref name="Schick 2002">Schick B, Plinkert PK, Prescher A. Aetiology of angiofibromas: reflection on their specific vascular component. Laryngo-rhino-otologie. 2002 Apr 1;81(4):280-4. https://doi.org/10.1055/s-2002-25322</ref> oncogenic mutations in C-MYC and C-KIT,<ref name="Pandey 2017">Pandey P, Mishra A, Tripathi AM, Verma V, Trivedi R, Singh HP, Kumar S, Patel B, Singh V, Pandey S, Pandey A. Current molecular profile of juvenile nasopharyngeal angiofibroma: first comprehensive study from India. The Laryngoscope. 2017 Mar;127(3):E100-6. https://doi.org/10.1002/lary.26250</ref> and a wide variety of other genetic aberrations.

===Epidemiology===
Juvenile nasopharyngeal angiofibroma has been reported to be the most common benign neoplasm of the nasopharynx in young males. It accounts for 0.05% to 0.5% of all head and neck tumors, with a reported incidence of 1 in 5,000 to 1 in 60,000 in the US annually.<ref name="Boghani 2013">Boghani Z, Husain Q, Kanumuri VV, Khan MN, Sangvhi S, Liu JK, Eloy JA. Juvenile nasopharyngeal angiofibroma: a systematic review and comparison of endoscopic, endoscopic‐assisted, and open resection in 1047 cases. The Laryngoscope. 2013 Apr;123(4):859-69. https://doi.org/10.1002/lary.23843</ref><ref name="Ferouz 1995">Ferouz AS, Mohr RM, Paul P. Juvenile nasopharyngeal angiofibroma and familial adenomatous polyposis: an association?. Otolaryngology—Head and Neck Surgery. 1995 Oct;113(4):435-9. https://doi.org/10.1016/S0194-59989570081-1</ref><ref name="Huang 2014">Huang Y, Liu Z, Wang J, Sun X, Yang L, Wang D. Surgical management of juvenile nasopharyngeal angiofibroma: analysis of 162 cases from 1995 to 2012. The Laryngoscope. 2014 Aug;124(8):1942-6. https://doi.org/10.1002/lary.24522</ref> It classically presents in adolescent males, with a typical age of presentation between 13 and 22 years old.<ref name ="Newman 2023">Newman M, Nguyen TB, McHugh T, Reddy K, Sommer DD. Early-onset juvenile nasopharyngeal angiofibroma (JNA): a systematic review. Journal of Otolaryngology-Head & Neck Surgery. 2023 Dec;52(1):s40463-023. https://doi.org/10.1186/s40463-023-00687-w</ref>

=== Genetics ===
There is no identified definitive genetic locus that predisposes patients to JNA's. However, there have been a number of studies looking at the various genetic components that could influence their formation.<ref name="Doody 2019">Doody J, Adil EA, Trenor III CC, Cunningham MJ. The genetic and molecular determinants of juvenile nasopharyngeal angiofibroma: a systematic review. Annals of Otology, Rhinology & Laryngology. 2019 Nov;128(11):1061-72. https://doi.org/10.1177/0003489419850194</ref> Due to fact that JNA's classically form in adolescent males, there have been several studies focused on the possible hormonal influence on their formation and growth. JNA's tend to have an abundance of progesterone and estradiol, but a relatively lower concentration of testosterone and dihydrotestosterone.<ref name="Kumagami 05 1993">Kumagami H. Estradiol, dihydrotestosterone, and testosterone in juvenile nasopharyngeal angiofibroma tissue. American Journal of Rhinology. 1993 May;7(3):101-4. https://doi.org/10.2500/105065893781976393</ref><ref name="Kumagami 01 1993">Kumagami H. Sex hormones in juvenile nasopharyngeal angiofibroma tissue. Auris Nasus Larynx. 1993 Jan 1;20(2):131-5. https://doi.org/10.1016/S0385-8146(12)80240-9</ref> Supplemental testosterone was trialed in JNA patients, but both tumor growth<ref name="Johnsen 1966">Johnsen S, Kloster JH, Schiff M. The action of hormones on juvenile nasopharyngeal angiofibroma. Acta Oto-Laryngologica. 1966 Jan 1;61(1-6):153-60. https://doi.org/10.3109/00016486609127052</ref> and tumor recurrence<ref name="Riggs 2010">Riggs S, Orlandi RR. Juvenile nasopharyngeal angiofibroma recurrence associated with exogenous testosterone therapy. Head & Neck: Journal for the Sciences and Specialties of the Head and Neck. 2010 Jun;32(6):812-5. https://doi.org/10.1002/hed.21152</ref> have been documented. Papers investigating potential targeted therapy markers have looked at steroid hormones, chromosomal abnormalities, growth factors, and other intracellular molecular targets.<ref name="Doody 2019"></ref> There have been studies investigating a possible link between Familial Adenomatous Polyposis (FAP) and JNA's via the adenomatous polyposis coli (APC)/β-catenin pathway, but no specific genetic link has been identified as of yet.<ref name="Guertl 2000">Guertl B, Beham A, Zechner R, Stammberger H, Hoefler G. Nasopharyngeal angiofibroma: an AM-Gene-Associated tumor?. Human pathology. 2000 Nov 1;31(11):1411-3.</ref>

=== Histology ===
Grossly on histologic section there are large fibrous/collagenous sections with fibroblasts, as well as vascular spaces of various sizes throughout the tumor.<ref name="Xu 2020">Xu B. Nasopharyngeal angiofibroma. PathologyOutlines.com website. https://www.pathologyoutlines.com/topic/nasalangiofibroma.html. Accessed November 17th, 2024.</ref> The stromal cells have an abundance of beta-catenin relative to the vascular endothelial cells.

<gallery>
JNA 30x.png|H&E stained section of green inked vascular channel (30x)
Beta Catenin JNA.png|Abnormal nuclear expression in stromal cells (blue arrow), compared with membranous / cytoplasmic staining in the endothelial cells (red arrow)
</gallery>

== Diagnosis ==
=== Patient History ===
Patients may have a history of any of the following symptoms:
* Unilateral nasal obstruction
* Recurrent epistaxis
* High-volume epistaxis
* Eustachian tube dysfunction
* Headache
* Facial swelling
* Anosmia / Hyposmia
* Cranial neuropathy
* Vision changes

=== Physical Examination ===
Many patients will not have obvious findings on anterior rhinoscopy. A large vascular-appearing mass is commonly fond on nasal endoscopy on the posterior lateral nasal sidewall near the region of the sphenopalatine artery. Note that '''''these hypervascular masses should not be biopsied''''', as this may result in significant bleeding that is hard to control in the clinic setting.

=== Laboratory Tests ===
Laboratory tests are not commonly useful in the diagnosis of JNA's, but should be performed preoperatively in the case of a planned surgical resection.
* Hemoglobin / Hematocrit
* Platelet level
* Platelet function (if family history of platelet dysfunction)
* PT / INR
* Blood Type and Screen (all patients)
* Blood Type and Cross (patients with advanced stage tumors where a significant blood loss is expected)

=== Imaging ===
[[File:Holman-Miller Sign.png|right|thumb|300 px|Axial CT scan of a JNA showing a right-sided Holman Miller sign]]
CT scan, angiography, and MRI are all useful in the workup of JNA's and have differing benefits.<ref name="Gaillard 2008">Gaillard F, Walizai T, Bell D, et al. Juvenile nasopharyngeal angiofibroma. Reference article, Radiopaedia.org (Accessed on 17 Nov 2024) https://doi.org/10.53347/rID-1541</ref> CT is typically the first imaging modality ordered in these patients, and is helpful for delineating the extent of bony erosion. Angiography demonstrates the extent of the mass well, and is frequently used in pre-operative embolization 24-48 hours before surgical resection. A majority of JNA's are supplied by the external carotid system, either by the maxillary artery (SPA) or the ascending pharyngeal artery.<ref name="Gaillard 2008"></ref> A lesser fraction of tumors are supplied by the internal carotid system (ophthalmic or sphenoidal branches), and this is typically in larger masses with intracranial invasion. MRI can be used to assess for intraorbital or intracranial extension. JNA's are T1 intermediate, T2 heterogenous with flow voids, and enhance with Gadolinium. The following are general characteristics often found in imaging of JNA's:
* Highly vascular nasopharyngeal mass
* Holman-Miller Sign<ref name="Niknejad 2013">Niknejad M, Tatco V, Bell D, et al. Holman-Miller sign (maxillary sinus). Reference article, Radiopaedia.org (Accessed on 17 Nov 2024) https://doi.org/10.53347/rID-21804</ref>: Anterior displacement of the posterior maxillary wall due to mass effect from the tumor
* Widening of the PMF or PPF
* Erosion of the pterygoid plate, especially the medial plate

There are several classification systems that have been proposed for JNAs based on imaging findings.

{| class="wikitable", style="margin-left: auto; margin-right: auto; border: none; text-align: center"
|+ Classification Systems for Juvenile Nasopharyngeal Angiofibromas
|-
! Classification System !! style=width:15em | Stage I !! style=width:15em | Stage II !! style=width:15em | Stage III !! style=width:15em | Stage IV !! style=width:15em | Stage V
|-
| Andrews (1989)<ref name="Andrews 1989">Andrews JC, Fisch U, Aeppli U, Valavanis A, Makek MS. The surgical management of extensive nasopharyngeal angiofibromas with the infratemporal fossa approach. The Laryngoscope. 1989 Apr;99(4):429-37. https://doi.org/10.1288/00005537-198904000-00013</ref> || Confined to NP || Invading one of the following with evidence of bony erosion: PPF, MS, ES, or SS || Invading ITF or orbit '''IIIa''': No intracranial extension '''IIIb''': Extradural (parasellar) extension || Intradural extension '''IVa''': No infiltration of CS, PF, or OC '''IVb''': Infiltration of CS, PF, or OC || --
|-
| Chandler (1984)<ref name="Chandler 1984">Chandler JR, Moskowitz L, Goulding R, Quencer RM. Nasopharyngeal angiofibromas: staging and management. Annals of Otology, Rhinology & Laryngology. 1984 Jul;93(4):322-9. https://doi.org/10.1177/000348948409300408</ref> || Confined to NP || Extension into the nasal cavity, SS, or both || Extension into any of the following: antrum, ES, PMF, ITF, orbit, or cheek || Intracranial extension || --
|-
| Onerci (2006)<ref name="Onerci 2006">Onerci M, Oğretmenoğlu O, Yücel T. Juvenile nasopharyngeal angiofibroma: a revised staging system. Rhinology. 2006 Mar 1;44(1):39-45. PMID: 16550949</ref>|| Nose, NP, ES, and SS or minimal extension into PMF || MS involvement, full occupation of PMF, extension to anterior cranial fossa, limited extension into ITF || Deep extension into cancellous bone at pterygoid base or body and GW of sphenoid, significant lateral extension into ITF or pterygoid plates, orbital involvement, CS obliteration || Intracranial extension between pituitary gland and ICA, tumor localization lateral to ICA, middle fossa extension and extensive intracranial extension || --
|-
| Radkowski (1996)<ref name="Radkowski 1996">Radkowski D, McGill T, Healy GB, Ohlms L, Jones DT. Angiofibroma: changes in staging and treatment. Archives of Otolaryngology–Head & Neck Surgery. 1996 Feb 1;122(2):122-9. https://doi.org/10.1001/archotol.1996.01890140012004</ref>|| '''IA''': Confined to nose or NP '''IB''': Extends into one or more sinuses || '''IIa''': Minimal extension into medial PMF '''IIb''': Full occupation of PMF with local mass effect '''IIc''': Extension into ITF, cheek, or posterior to pterygoid plates || Erosion of skull base '''IIIa''': Minimal skull base involvement '''IIIb''': Extensive intracranial extension, with or without invasion into CS || -- || --
|-
| Sessions (1981)<ref name="Sessions 1981">Sessions RB, Bryan RN, Naclerio RM, Alford BR. Radiographic staging of juvenile angiofibroma. Head & neck surgery. 1981 Mar;3(4):279-83. https://doi.org/10.1002/hed.2890030404</ref> || '''IA''': Confined to nose or NP '''IB''': Extends into one or more sinuses || '''IIa''': Minimal extension into PMF '''IIb''': Full occupation of PMF with or without orbital erosion '''IIc''': ITF with or without cheek extension || Intracranial extension || -- || --
|-
| UPMC (2010)<ref name="Snyderman 2010">Snyderman CH, Pant H, Carrau RL, Gardner P. A new endoscopic staging system for angiofibromas. Archives of Otolaryngology–Head & Neck Surgery. 2010 Jun 21;136(6):588-94. https://doi.org/10.1001/archoto.2010.83</ref> || Nasal cavity, medial PPF || Paranasal sinuses, lateral PPF; no residual vascularity || Skull base erosion, orbit, ITF involvement; no residual vascularity || Skull base erosion, orbit, ITF involvement; residual vascularity || Intracranial extension with residual vascularity '''M''': Medial extension '''L''': Lateral extension
|-
|+ style="caption-side:bottom; font-weight: normal;"|''Abbreviations: Cavernous sinus (CS), Ethmoid sinus (ES), Infratemporal fossa (ITF), Internal carotid artery (ICA), Maxillary sinus (MS), Nasopharynx (NP), Optic chiasm (OC), Pituitary fossa (PF), Pterygomaxillary fissure (PMF), Pterygopalatine fossa (PPF), Sphenoid sinus (SS)''
|}

=== Differential Diagnosis ===
In the case of a nasopharyngeal mass, the following should also be considered:
* Clival chondroma
* Encephalocele
* Esthesioneuroblastoma
* Inverted papilloma
* Nasal polyp
* Nasopharyngeal carcinoma
* Nasopharyngeal teratoma
* Rhabdomyosarcoma
* Vascular malformation

== Management ==
=== Medical Management ===
There is no approved medical treatment for management of juvenile nasopharyngeal angiofibromas. The treatment is primarily surgical. Medical management focuses on optimizing patients before surgery, such as addressing any coagulopathies or addressing underlying anemia.

=== Surgical Management ===
Historically, surgical management of JNAs involved aggressive open approaches, including transpalatal, lateral rhinotomy, midface degloving, or infratemporal fossa craniotomy approaches.<ref name="Renkonen 2011">Renkonen S, Hagström J, Vuola J, Niemelä M, Porras M, Kivivuori SM, Leivo I, Mäkitie AA. The changing surgical management of juvenile nasopharyngeal angiofibroma. European archives of oto-rhino-laryngology. 2011 Apr;268:599-607. https://doi.org/10.1007/s00405-010-1383-z</ref> In recent years, there has been a transition towards transnasal endoscopic approaches. This has resulted in better visualization during surgery and a subsequent improvement in intraoperative blood loss, need for blood transfusions,<ref name="Oliveira 2012">Oliveira JA, Tavares MG, Aguiar CV, Azevedo JF, Sousa JR, Almeida PC, Gomes EF. Comparison between endoscopic and open surgery in 37 patients with nasopharyngeal angiofibroma. Brazilian Journal of otorhinolaryngology. 2012;78:75-80. https://doi.org/10.1590/S1808-86942012000100012</ref> and recurrence rates.<ref name="Bosraty 2011">Bosraty H, Atef A, Aziz M. Endoscopic vs. open surgery for treating large, locally advanced juvenile angiofibromas: A comparison of local control and morbidity outcomes. ENT: Ear, Nose & Throat Journal. 2011 Nov 1;90(11). PMID: 22109921</ref> Prior to surgery, preoperative embolization should be considered to limit blood loss intraoperatively.

== Outcomes ==
=== Complications ===
The primary concerns in the surgical management of juvenile nasopharyngeal angiofibroma are bleeding and recurrence rates. Iatrogenic injuries to structures of the orbit (such as ophthalmoplegia) and skull base (such as facial paresthesias) are also a concern depending on the degree of tumor extension.

==== Bleeding ====
Intraoperative bleeding rates vary based on technique, whether preoperative embolization was used, and tumor staging. Endoscopic surgery is generally accepted to have lower intraoperative blood loss relative to more traditional open approaches.<ref name="Oliveira 2012"></ref> Preoperative embolization has also been found to significantly lower intraoperative blood loss. One study of 47 patients (primarily Radkowski stages I-II) found that patients who underwent preoperative embolization had a mean estimated blood loss (EBL) of 770 mL, and patients who did not undergo embolization had a mean EBL of more than 1,400 mL.<ref name="Ardehali 2010">Ardehali MM, Ardestani SH, Yazdani N, Goodarzi H, Bastaninejad S. Endoscopic approach for excision of juvenile nasopharyngeal angiofibroma: complications and outcomes. American journal of otolaryngology. 2010 Sep 1;31(5):343-9. https://doi.org/10.1016/j.amjoto.2009.04.007</ref> The same study noted that embolized patients had a mean hospital stay of 1.8 days, vs 3.1 days for all patients in the study. A systematic review of advance-stage JNAs with intracranial extension found a mean EBL of 1,700 mL and a similar reduction in EBL following preoperative embolization.<ref name="Leong 2013">Leong SC. A systematic review of surgical outcomes for advanced juvenile nasopharyngeal angiofibroma with intracranial involvement. The Laryngoscope. 2013 May;123(5):1125-31. https://doi.org/10.1002/lary.23760</ref>

==== Recurrence ====
Recurrence rates are similarly related to surgical technique and tumor stage. Endoscopic approaches have been documented to have an improved recurrence rate relative to open approaches.<ref name="Bosraty 2011"></ref> A systematic review of 92 studies totaling 821 patients found a recurrence rate of about 10%, as well as 7.7% with residual tumor.<ref name="Khoueir 2013">Khoueir N, Nicolas N, Rohayem Z, Haddad A, Abou Hamad W. Exclusive endoscopic resection of juvenile nasopharyngeal angiofibroma: a systematic review of the literature. Otolaryngology–Head and Neck Surgery. 2014 Mar;150(3):350-8. https://doi.org/10.1177/0194599813516605</ref> Studies looking at more invasive tumors have a higher rate of recurrence of approximately 18%.<ref name="Leong 2013"></ref>

=== Prognosis ===
Recurrence rates can be very high and increase with increased stage at the time of surgery. Recurrence has been reported from 22%<ref name="Bleier 2009">Bleier BS, Kennedy DW, Palmer JN, Chiu AG, Bloom JD, O'Malley Jr BW. Current management of juvenile nasopharyngeal angiofibroma: a tertiary center experience 1999–2007. American journal of rhinology & allergy. 2009 May;23(3):328-30. https://doi.org/10.2500/ajra.2009.23.3322</ref> to as high as 61% in advanced disease.<ref name="Huang 2014">Huang Y, Liu Z, Wang J, Sun X, Yang L, Wang D. Surgical management of juvenile nasopharyngeal angiofibroma: analysis of 162 cases from 1995 to 2012. The Laryngoscope. 2014 Aug;124(8):1942-6. https://doi.org/10.1002/lary.24522</ref>

== References ==
<references />

Juvenile Nasopharyngeal Angiofibroma

2024-11-18T16:29:17Z

Jack.Dewey:

{{infobox Disease
|Title =
|Aliases =
|Image = [[File:Nasopharyngeal angiofibroma - 2 - high mag.jpg]]
|Caption = Histologic section of a JNA
|ICD-9 = 210.7
|ICD-10 = D10.6
|MeSH =
|Gene =
|Locus =
|OMIM =
|EyeWiki =
|Radiopaedia = [https://radiopaedia.org/articles/juvenile-nasopharyngeal-angiofibroma?lang=us Juvenile Nasopharyngeal Angiofibroma]
|Pathology = [https://www.pathologyoutlines.com/topic/nasalangiofibroma.html Nasal Angiofibroma]
}}

== Overview ==
'''Juvenile nasopharyngeal angiofibroma (JNA)''' is a benign vascular neoplasm of the nasal cavity and nasopharynx that classically presents in adolescent boys.

== Pathophysiology ==
=== Relevant Anatomy ===
JNA's typically originate in the region of the sphenopalatine artery (SPA) in the posterior nasal cavity/nasopharynx. From there, they can extend into the paranasal sinuses, pterygopalatine fossa (PPF), infratemporal fossa (ITF), orbit, or cranial vault.

<gallery>
Pterygopalatine fossa.jpg|Cadaveric model of the PPF
Schematic diagram showing the bony anatomy and main neural connections of the PPF.png|Neural contents of the PPF
Gray511.png|Maxillary artery branches, with SPA at the distal end
Gerrish's Text-book of Anatomy (1902) - Fig. 243.png|Location of the sphenopalatine foramen
Basilar process and palatine process.jpg|Location of the ITF
</gallery>

=== Disease Etiology ===
JNA's are fibrovascular tumors that arise from the posterior lateral nasal cavity near the choana. There is substantial debate about the exact circumstances and location that lead to the formation of a JNA, but they are generally accepted to form near the region of the sphenopalatine foramen. Proposed theories regarding their etiology include androgen receptor-related growth,<ref name="Liu 2015">Liu Z, Wang J, Wang H, Wang D, Hu L, Liu Q, Sun X. Hormonal receptors and vascular endothelial growth factor in juvenile nasopharyngeal angiofibroma: immunohistochemical and tissue microarray analysis. Acta oto-laryngologica. 2015 Jan 2;135(1):51-7. https://doi.org/10.3109/00016489.2014.952774</ref> incomplete regression of a branchial artery during embryogenesis,<ref name="Schick 2002">Schick B, Plinkert PK, Prescher A. Aetiology of angiofibromas: reflection on their specific vascular component. Laryngo-rhino-otologie. 2002 Apr 1;81(4):280-4. https://doi.org/10.1055/s-2002-25322</ref> oncogenic mutations in C-MYC and C-KIT,<ref name="Pandey 2017">Pandey P, Mishra A, Tripathi AM, Verma V, Trivedi R, Singh HP, Kumar S, Patel B, Singh V, Pandey S, Pandey A. Current molecular profile of juvenile nasopharyngeal angiofibroma: first comprehensive study from India. The Laryngoscope. 2017 Mar;127(3):E100-6. https://doi.org/10.1002/lary.26250</ref> and a wide variety of other genetic aberrations.

===Epidemiology===
Juvenile nasopharyngeal angiofibroma has been reported to be the most common benign neoplasm of the nasopharynx in young males. It accounts for 0.05% to 0.5% of all head and neck tumors, with a reported incidence of 1 in 5,000 to 1 in 60,000 in the US annually.<ref name="Boghani 2013">Boghani Z, Husain Q, Kanumuri VV, Khan MN, Sangvhi S, Liu JK, Eloy JA. Juvenile nasopharyngeal angiofibroma: a systematic review and comparison of endoscopic, endoscopic‐assisted, and open resection in 1047 cases. The Laryngoscope. 2013 Apr;123(4):859-69. https://doi.org/10.1002/lary.23843</ref><ref name="Ferouz 1995">Ferouz AS, Mohr RM, Paul P. Juvenile nasopharyngeal angiofibroma and familial adenomatous polyposis: an association?. Otolaryngology—Head and Neck Surgery. 1995 Oct;113(4):435-9. https://doi.org/10.1016/S0194-59989570081-1</ref><ref name="Huang 2014">Huang Y, Liu Z, Wang J, Sun X, Yang L, Wang D. Surgical management of juvenile nasopharyngeal angiofibroma: analysis of 162 cases from 1995 to 2012. The Laryngoscope. 2014 Aug;124(8):1942-6. https://doi.org/10.1002/lary.24522</ref> It classically presents in adolescent males, with a typical age of presentation between 13 and 22 years old.<ref name ="Newman 2023">Newman M, Nguyen TB, McHugh T, Reddy K, Sommer DD. Early-onset juvenile nasopharyngeal angiofibroma (JNA): a systematic review. Journal of Otolaryngology-Head & Neck Surgery. 2023 Dec;52(1):s40463-023. https://doi.org/10.1186/s40463-023-00687-w</ref>

=== Genetics ===
There is no identified definitive genetic locus that predisposes patients to JNA's. However, there have been a number of studies looking at the various genetic components that could influence their formation.<ref name="Doody 2019">Doody J, Adil EA, Trenor III CC, Cunningham MJ. The genetic and molecular determinants of juvenile nasopharyngeal angiofibroma: a systematic review. Annals of Otology, Rhinology & Laryngology. 2019 Nov;128(11):1061-72. https://doi.org/10.1177/0003489419850194</ref> Due to fact that JNA's classically form in adolescent males, there have been several studies focused on the possible hormonal influence on their formation and growth. JNA's tend to have an abundance of progesterone and estradiol, but a relatively lower concentration of testosterone and dihydrotestosterone.<ref name="Kumagami 05 1993">Kumagami H. Estradiol, dihydrotestosterone, and testosterone in juvenile nasopharyngeal angiofibroma tissue. American Journal of Rhinology. 1993 May;7(3):101-4. https://doi.org/10.2500/105065893781976393</ref><ref name="Kumagami 01 1993">Kumagami H. Sex hormones in juvenile nasopharyngeal angiofibroma tissue. Auris Nasus Larynx. 1993 Jan 1;20(2):131-5. https://doi.org/10.1016/S0385-8146(12)80240-9</ref> Supplemental testosterone was trialed in JNA patients, but both tumor growth<ref name="Johnsen 1966">Johnsen S, Kloster JH, Schiff M. The action of hormones on juvenile nasopharyngeal angiofibroma. Acta Oto-Laryngologica. 1966 Jan 1;61(1-6):153-60. https://doi.org/10.3109/00016486609127052</ref> and tumor recurrence<ref name="Riggs 2010">Riggs S, Orlandi RR. Juvenile nasopharyngeal angiofibroma recurrence associated with exogenous testosterone therapy. Head & Neck: Journal for the Sciences and Specialties of the Head and Neck. 2010 Jun;32(6):812-5. https://doi.org/10.1002/hed.21152</ref> have been documented. Papers investigating potential targeted therapy markers have looked at steroid hormones, chromosomal abnormalities, growth factors, and other intracellular molecular targets.<ref name="Doody 2019"></ref> There have been studies investigating a possible link between Familial Adenomatous Polyposis (FAP) and JNA's via the adenomatous polyposis coli (APC)/β-catenin pathway, but no specific genetic link has been identified as of yet.<ref name="Guertl 2000">Guertl B, Beham A, Zechner R, Stammberger H, Hoefler G. Nasopharyngeal angiofibroma: an AM-Gene-Associated tumor?. Human pathology. 2000 Nov 1;31(11):1411-3.</ref>

=== Histology ===
Grossly on histologic section there are large fibrous/collagenous sections with fibroblasts, as well as vascular spaces of various sizes throughout the tumor.<ref name="Xu 2020">Xu B. Nasopharyngeal angiofibroma. PathologyOutlines.com website. https://www.pathologyoutlines.com/topic/nasalangiofibroma.html. Accessed November 17th, 2024.</ref> The stromal cells have an abundance of beta-catenin relative to the vascular endothelial cells.

<gallery>
JNA 30x.png|H&E stained section of green inked vascular channel (30x)
Beta Catenin JNA.png|Abnormal nuclear expression in stromal cells (blue arrow), compared with membranous / cytoplasmic staining in the endothelial cells (red arrow)
</gallery>

== Diagnosis ==
=== Patient History ===
Patients may have a history of any of the following symptoms:
* Unilateral nasal obstruction
* Recurrent epistaxis
* High-volume epistaxis
* Eustachian tube dysfunction
* Headache
* Facial swelling
* Anosmia / Hyposmia
* Cranial neuropathy
* Vision changes

=== Physical Examination ===
Many patients will not have obvious findings on anterior rhinoscopy. A large vascular-appearing mass is commonly fond on nasal endoscopy on the posterior lateral nasal sidewall near the region of the sphenopalatine artery. Note that '''''these hypervascular masses should not be biopsied''''', as this may result in significant bleeding that is hard to control in the clinic setting.

=== Laboratory Tests ===
Laboratory tests are not commonly useful in the diagnosis of JNA's, but should be performed preoperatively in the case of a planned surgical resection.
* Hemoglobin / Hematocrit
* Platelet level
* Platelet function (if family history of platelet dysfunction)
* PT / INR
* Blood Type and Screen (all patients)
* Blood Type and Cross (patients with advanced stage tumors where a significant blood loss is expected)

=== Imaging ===
[[File:Holman-Miller Sign.png|right|thumb|300 px|Axial CT scan of a JNA showing a right-sided Holman Miller sign]]
CT scan, angiography, and MRI are all useful in the workup of JNA's and have differing benefits.<ref name="Gaillard 2008">Gaillard F, Walizai T, Bell D, et al. Juvenile nasopharyngeal angiofibroma. Reference article, Radiopaedia.org (Accessed on 17 Nov 2024) https://doi.org/10.53347/rID-1541</ref> CT is typically the first imaging modality ordered in these patients, and is helpful for delineating the extent of bony erosion. Angiography demonstrates the extent of the mass well, and is frequently used in pre-operative embolization 24-48 hours before surgical resection. A majority of JNA's are supplied by the external carotid system, either by the maxillary artery (SPA) or the ascending pharyngeal artery.<ref name="Gaillard 2008"></ref> A lesser fraction of tumors are supplied by the internal carotid system (ophthalmic or sphenoidal branches), and this is typically in larger masses with intracranial invasion. MRI can be used to assess for intraorbital or intracranial extension. JNA's are T1 intermediate, T2 heterogenous with flow voids, and enhance with Gadolinium. The following are general characteristics often found in imaging of JNA's:
* Highly vascular nasopharyngeal mass
* Holman-Miller Sign<ref name="Niknejad 2013">Niknejad M, Tatco V, Bell D, et al. Holman-Miller sign (maxillary sinus). Reference article, Radiopaedia.org (Accessed on 17 Nov 2024) https://doi.org/10.53347/rID-21804</ref>: Anterior displacement of the posterior maxillary wall due to mass effect from the tumor
* Widening of the PMF or PPF
* Erosion of the pterygoid plate, especially the medial plate

There are several classification systems that have been proposed for JNAs based on imaging findings.

{| class="wikitable", style="margin-left: auto; margin-right: auto; border: none; text-align: center"
|+ Classification Systems for Juvenile Nasopharyngeal Angiofibromas
|-
! Classification System !! style=width:15em | Stage I !! style=width:15em | Stage II !! style=width:15em | Stage III !! style=width:15em | Stage IV !! style=width:15em | Stage V
|-
| Andrews (1989)<ref name="Andrews 1989">Andrews JC, Fisch U, Aeppli U, Valavanis A, Makek MS. The surgical management of extensive nasopharyngeal angiofibromas with the infratemporal fossa approach. The Laryngoscope. 1989 Apr;99(4):429-37. https://doi.org/10.1288/00005537-198904000-00013</ref> || Confined to NP || Invading one of the following with evidence of bony erosion: PPF, MS, ES, or SS || Invading ITF or orbit '''IIIa''': No intracranial extension '''IIIb''': Extradural (parasellar) extension || Intradural extension '''IVa''': No infiltration of CS, PF, or OC '''IVb''': Infiltration of CS, PF, or OC || --
|-
| Chandler (1984)<ref name="Chandler 1984">Chandler JR, Moskowitz L, Goulding R, Quencer RM. Nasopharyngeal angiofibromas: staging and management. Annals of Otology, Rhinology & Laryngology. 1984 Jul;93(4):322-9. https://doi.org/10.1177/000348948409300408</ref> || Confined to NP || Extension into the nasal cavity, SS, or both || Extension into any of the following: antrum, ES, PMF, ITF, orbit, or cheek || Intracranial extension || --
|-
| Onerci (2006)<ref name="Onerci 2006">Onerci M, Oğretmenoğlu O, Yücel T. Juvenile nasopharyngeal angiofibroma: a revised staging system. Rhinology. 2006 Mar 1;44(1):39-45. PMID: 16550949</ref>|| Nose, NP, ES, and SS or minimal extension into PMF || MS involvement, full occupation of PMF, extension to anterior cranial fossa, limited extension into ITF || Deep extension into cancellous bone at pterygoid base or body and GW of sphenoid, significant lateral extension into ITF or pterygoid plates, orbital involvement, CS obliteration || Intracranial extension between pituitary gland and ICA, tumor localization lateral to ICA, middle fossa extension and extensive intracranial extension || --
|-
| Radkowski (1996)<ref name="Radkowski 1996">Radkowski D, McGill T, Healy GB, Ohlms L, Jones DT. Angiofibroma: changes in staging and treatment. Archives of Otolaryngology–Head & Neck Surgery. 1996 Feb 1;122(2):122-9. https://doi.org/10.1001/archotol.1996.01890140012004</ref>|| '''IA''': Confined to nose or NP '''IB''': Extends into one or more sinuses || '''IIa''': Minimal extension into medial PMF '''IIb''': Full occupation of PMF with local mass effect '''IIc''': Extension into ITF, cheek, or posterior to pterygoid plates || Erosion of skull base '''IIIa''': Minimal skull base involvement '''IIIb''': Extensive intracranial extension, with or without invasion into CS || -- || --
|-
| Sessions (1981)<ref name="Sessions 1981">Sessions RB, Bryan RN, Naclerio RM, Alford BR. Radiographic staging of juvenile angiofibroma. Head & neck surgery. 1981 Mar;3(4):279-83. https://doi.org/10.1002/hed.2890030404</ref> || '''IA''': Confined to nose or NP '''IB''': Extends into one or more sinuses || '''IIa''': Minimal extension into PMF '''IIb''': Full occupation of PMF with or without orbital erosion '''IIc''': ITF with or without cheek extension || Intracranial extension || -- || --
|-
| UPMC (2010)<ref name="Snyderman 2010">Snyderman CH, Pant H, Carrau RL, Gardner P. A new endoscopic staging system for angiofibromas. Archives of Otolaryngology–Head & Neck Surgery. 2010 Jun 21;136(6):588-94. https://doi.org/10.1001/archoto.2010.83</ref> || Nasal cavity, medial PPF || Paranasal sinuses, lateral PPF; no residual vascularity || Skull base erosion, orbit, ITF involvement; no residual vascularity || Skull base erosion, orbit, ITF involvement; residual vascularity || Intracranial extension with residual vascularity '''M''': Medial extension '''L''': Lateral extension
|-
|+ style="caption-side:bottom; font-weight: normal;"|''Abbreviations: Cavernous sinus (CS), Ethmoid sinus (ES), Infratemporal fossa (ITF), Internal carotid artery (ICA), Maxillary sinus (MS), Nasopharynx (NP), Optic chiasm (OC), Pituitary fossa (PF), Pterygomaxillary fissure (PMF), Pterygopalatine fossa (PPF), Sphenoid sinus (SS)''
|}

=== Differential Diagnosis ===
In the case of a nasopharyngeal mass, the following should also be considered:
* Clival chondroma
* Encephalocele
* Esthesioneuroblastoma
* Inverted papilloma
* Nasal polyp
* Nasopharyngeal carcinoma
* Nasopharyngeal teratoma
* Rhabdomyosarcoma
* Vascular malformation

== Management ==
=== Medical Management ===
There is no approved medical treatment for management of juvenile nasopharyngeal angiofibromas. The treatment is primarily surgical. Medical management focuses on optimizing patients before surgery, such as addressing any coagulopathies or addressing underlying anemia.

=== Surgical Management ===
Historically, surgical management of JNAs involved aggressive open approaches, including transpalatal, lateral rhinotomy, midface degloving, or infratemporal fossa craniotomy approaches.<ref name="Renkonen 2011">Renkonen S, Hagström J, Vuola J, Niemelä M, Porras M, Kivivuori SM, Leivo I, Mäkitie AA. The changing surgical management of juvenile nasopharyngeal angiofibroma. European archives of oto-rhino-laryngology. 2011 Apr;268:599-607. https://doi.org/10.1007/s00405-010-1383-z</ref> In recent years, there has been a transition towards transnasal endoscopic approaches. This has resulted in better visualization during surgery and a subsequent improvement in intraoperative blood loss, need for blood transfusions,<ref name="Oliveira 2012">Oliveira JA, Tavares MG, Aguiar CV, Azevedo JF, Sousa JR, Almeida PC, Gomes EF. Comparison between endoscopic and open surgery in 37 patients with nasopharyngeal angiofibroma. Brazilian Journal of otorhinolaryngology. 2012;78:75-80. https://doi.org/10.1590/S1808-86942012000100012</ref> and recurrence rates.<ref name="Bosraty 2011">Bosraty H, Atef A, Aziz M. Endoscopic vs. open surgery for treating large, locally advanced juvenile angiofibromas: A comparison of local control and morbidity outcomes. ENT: Ear, Nose & Throat Journal. 2011 Nov 1;90(11). PMID: 22109921</ref> Prior to surgery, preoperative embolization should be considered to limit blood loss intraoperatively.

== Outcomes ==
=== Complications ===
The primary concerns in the surgical management of juvenile nasopharyngeal angiofibroma are bleeding and recurrence rates. Iatrogenic injuries to structures of the orbit (such as ophthalmoplegia) and skull base (such as facial paresthesias) are also a concern depending on the degree of tumor extension.

==== Bleeding ====
Intraoperative bleeding rates vary based on technique, whether preoperative embolization was used, and tumor staging. Endoscopic surgery is generally accepted to have lower intraoperative blood loss relative to more traditional open approaches.<ref name="Oliveira 2012"></ref> Preoperative embolization has also been found to significantly lower intraoperative blood loss. One study of 47 patients (primarily Radkowski stages I-II) found that patients who underwent preoperative embolization had a mean estimated blood loss (EBL) of 770 mL, and patients who did not undergo embolization had a mean EBL of more than 1,400 mL.<ref name="Ardehali 2010">Ardehali MM, Ardestani SH, Yazdani N, Goodarzi H, Bastaninejad S. Endoscopic approach for excision of juvenile nasopharyngeal angiofibroma: complications and outcomes. American journal of otolaryngology. 2010 Sep 1;31(5):343-9. https://doi.org/10.1016/j.amjoto.2009.04.007</ref> The same study noted that embolized patients had a mean hospital stay of 1.8 days, vs 3.1 days for all patients in the study. A systematic review of advance-stage JNAs with intracranial extension found a mean EBL of 1,700 mL and a similar reduction in EBL following preoperative embolization.<ref name="Leong 2013">Leong SC. A systematic review of surgical outcomes for advanced juvenile nasopharyngeal angiofibroma with intracranial involvement. The Laryngoscope. 2013 May;123(5):1125-31. https://doi.org/10.1002/lary.23760</ref>

==== Recurrence ====
Recurrence rates are similarly related to surgical technique and tumor stage. Endoscopic approaches have been documented to have an improved recurrence rate relative to open approaches.<ref name="Bosraty 2011"></ref> A systematic review of 92 studies totaling 821 patients found a recurrence rate of about 10% and 7.7% with residual tumor.*** Studies looking at more invasive tumors have a higher rate of recurrence

=== Prognosis ===
Recurrence rates can be very high and increase with increased stage at the time of surgery. Recurrence has been reported from 22%<ref name="Bleier 2009">Bleier BS, Kennedy DW, Palmer JN, Chiu AG, Bloom JD, O'Malley Jr BW. Current management of juvenile nasopharyngeal angiofibroma: a tertiary center experience 1999–2007. American journal of rhinology & allergy. 2009 May;23(3):328-30. https://doi.org/10.2500/ajra.2009.23.3322</ref> to as high as 61% in advanced disease.<ref name="Huang 2014">Huang Y, Liu Z, Wang J, Sun X, Yang L, Wang D. Surgical management of juvenile nasopharyngeal angiofibroma: analysis of 162 cases from 1995 to 2012. The Laryngoscope. 2014 Aug;124(8):1942-6. https://doi.org/10.1002/lary.24522</ref>

== References ==
<references />

Juvenile Nasopharyngeal Angiofibroma

2024-11-18T16:20:03Z

Jack.Dewey:

{{infobox Disease
|Title =
|Aliases =
|Image = [[File:Nasopharyngeal angiofibroma - 2 - high mag.jpg]]
|Caption = Histologic section of a JNA
|ICD-9 = 210.7
|ICD-10 = D10.6
|MeSH =
|Gene =
|Locus =
|OMIM =
|EyeWiki =
|Radiopaedia = [https://radiopaedia.org/articles/juvenile-nasopharyngeal-angiofibroma?lang=us Juvenile Nasopharyngeal Angiofibroma]
|Pathology = [https://www.pathologyoutlines.com/topic/nasalangiofibroma.html Nasal Angiofibroma]
}}

== Overview ==
'''Juvenile nasopharyngeal angiofibroma (JNA)''' is a benign vascular neoplasm of the nasal cavity and nasopharynx that classically presents in adolescent boys.

== Pathophysiology ==
=== Relevant Anatomy ===
JNA's typically originate in the region of the sphenopalatine artery (SPA) in the posterior nasal cavity/nasopharynx. From there, they can extend into the paranasal sinuses, pterygopalatine fossa (PPF), infratemporal fossa (ITF), orbit, or cranial vault.

<gallery>
Pterygopalatine fossa.jpg|Cadaveric model of the PPF
Schematic diagram showing the bony anatomy and main neural connections of the PPF.png|Neural contents of the PPF
Gray511.png|Maxillary artery branches, with SPA at the distal end
Gerrish's Text-book of Anatomy (1902) - Fig. 243.png|Location of the sphenopalatine foramen
Basilar process and palatine process.jpg|Location of the ITF
</gallery>

=== Disease Etiology ===
JNA's are fibrovascular tumors that arise from the posterior lateral nasal cavity near the choana. There is substantial debate about the exact circumstances and location that lead to the formation of a JNA, but they are generally accepted to form near the region of the sphenopalatine foramen. Proposed theories regarding their etiology include androgen receptor-related growth,<ref name="Liu 2015">Liu Z, Wang J, Wang H, Wang D, Hu L, Liu Q, Sun X. Hormonal receptors and vascular endothelial growth factor in juvenile nasopharyngeal angiofibroma: immunohistochemical and tissue microarray analysis. Acta oto-laryngologica. 2015 Jan 2;135(1):51-7. https://doi.org/10.3109/00016489.2014.952774</ref> incomplete regression of a branchial artery during embryogenesis,<ref name="Schick 2002">Schick B, Plinkert PK, Prescher A. Aetiology of angiofibromas: reflection on their specific vascular component. Laryngo-rhino-otologie. 2002 Apr 1;81(4):280-4. https://doi.org/10.1055/s-2002-25322</ref> oncogenic mutations in C-MYC and C-KIT,<ref name="Pandey 2017">Pandey P, Mishra A, Tripathi AM, Verma V, Trivedi R, Singh HP, Kumar S, Patel B, Singh V, Pandey S, Pandey A. Current molecular profile of juvenile nasopharyngeal angiofibroma: first comprehensive study from India. The Laryngoscope. 2017 Mar;127(3):E100-6. https://doi.org/10.1002/lary.26250</ref> and a wide variety of other genetic aberrations.

===Epidemiology===
Juvenile nasopharyngeal angiofibroma has been reported to be the most common benign neoplasm of the nasopharynx in young males. It accounts for 0.05% to 0.5% of all head and neck tumors, with a reported incidence of 1 in 5,000 to 1 in 60,000 in the US annually.<ref name="Boghani 2013">Boghani Z, Husain Q, Kanumuri VV, Khan MN, Sangvhi S, Liu JK, Eloy JA. Juvenile nasopharyngeal angiofibroma: a systematic review and comparison of endoscopic, endoscopic‐assisted, and open resection in 1047 cases. The Laryngoscope. 2013 Apr;123(4):859-69. https://doi.org/10.1002/lary.23843</ref><ref name="Ferouz 1995">Ferouz AS, Mohr RM, Paul P. Juvenile nasopharyngeal angiofibroma and familial adenomatous polyposis: an association?. Otolaryngology—Head and Neck Surgery. 1995 Oct;113(4):435-9. https://doi.org/10.1016/S0194-59989570081-1</ref><ref name="Huang 2014">Huang Y, Liu Z, Wang J, Sun X, Yang L, Wang D. Surgical management of juvenile nasopharyngeal angiofibroma: analysis of 162 cases from 1995 to 2012. The Laryngoscope. 2014 Aug;124(8):1942-6. https://doi.org/10.1002/lary.24522</ref> It classically presents in adolescent males, with a typical age of presentation between 13 and 22 years old.<ref name ="Newman 2023">Newman M, Nguyen TB, McHugh T, Reddy K, Sommer DD. Early-onset juvenile nasopharyngeal angiofibroma (JNA): a systematic review. Journal of Otolaryngology-Head & Neck Surgery. 2023 Dec;52(1):s40463-023. https://doi.org/10.1186/s40463-023-00687-w</ref>

=== Genetics ===
There is no identified definitive genetic locus that predisposes patients to JNA's. However, there have been a number of studies looking at the various genetic components that could influence their formation.<ref name="Doody 2019">Doody J, Adil EA, Trenor III CC, Cunningham MJ. The genetic and molecular determinants of juvenile nasopharyngeal angiofibroma: a systematic review. Annals of Otology, Rhinology & Laryngology. 2019 Nov;128(11):1061-72. https://doi.org/10.1177/0003489419850194</ref> Due to fact that JNA's classically form in adolescent males, there have been several studies focused on the possible hormonal influence on their formation and growth. JNA's tend to have an abundance of progesterone and estradiol, but a relatively lower concentration of testosterone and dihydrotestosterone.<ref name="Kumagami 05 1993">Kumagami H. Estradiol, dihydrotestosterone, and testosterone in juvenile nasopharyngeal angiofibroma tissue. American Journal of Rhinology. 1993 May;7(3):101-4. https://doi.org/10.2500/105065893781976393</ref><ref name="Kumagami 01 1993">Kumagami H. Sex hormones in juvenile nasopharyngeal angiofibroma tissue. Auris Nasus Larynx. 1993 Jan 1;20(2):131-5. https://doi.org/10.1016/S0385-8146(12)80240-9</ref> Supplemental testosterone was trialed in JNA patients, but both tumor growth<ref name="Johnsen 1966">Johnsen S, Kloster JH, Schiff M. The action of hormones on juvenile nasopharyngeal angiofibroma. Acta Oto-Laryngologica. 1966 Jan 1;61(1-6):153-60. https://doi.org/10.3109/00016486609127052</ref> and tumor recurrence<ref name="Riggs 2010">Riggs S, Orlandi RR. Juvenile nasopharyngeal angiofibroma recurrence associated with exogenous testosterone therapy. Head & Neck: Journal for the Sciences and Specialties of the Head and Neck. 2010 Jun;32(6):812-5. https://doi.org/10.1002/hed.21152</ref> have been documented. Papers investigating potential targeted therapy markers have looked at steroid hormones, chromosomal abnormalities, growth factors, and other intracellular molecular targets.<ref name="Doody 2019"></ref> There have been studies investigating a possible link between Familial Adenomatous Polyposis (FAP) and JNA's via the adenomatous polyposis coli (APC)/β-catenin pathway, but no specific genetic link has been identified as of yet.<ref name="Guertl 2000">Guertl B, Beham A, Zechner R, Stammberger H, Hoefler G. Nasopharyngeal angiofibroma: an AM-Gene-Associated tumor?. Human pathology. 2000 Nov 1;31(11):1411-3.</ref>

=== Histology ===
Grossly on histologic section there are large fibrous/collagenous sections with fibroblasts, as well as vascular spaces of various sizes throughout the tumor.<ref name="Xu 2020">Xu B. Nasopharyngeal angiofibroma. PathologyOutlines.com website. https://www.pathologyoutlines.com/topic/nasalangiofibroma.html. Accessed November 17th, 2024.</ref> The stromal cells have an abundance of beta-catenin relative to the vascular endothelial cells.

<gallery>
JNA 30x.png|H&E stained section of green inked vascular channel (30x)
Beta Catenin JNA.png|Abnormal nuclear expression in stromal cells (blue arrow), compared with membranous / cytoplasmic staining in the endothelial cells (red arrow)
</gallery>

== Diagnosis ==
=== Patient History ===
Patients may have a history of any of the following symptoms:
* Unilateral nasal obstruction
* Recurrent epistaxis
* High-volume epistaxis
* Eustachian tube dysfunction
* Headache
* Facial swelling
* Anosmia / Hyposmia
* Cranial neuropathy
* Vision changes

=== Physical Examination ===
Many patients will not have obvious findings on anterior rhinoscopy. A large vascular-appearing mass is commonly fond on nasal endoscopy on the posterior lateral nasal sidewall near the region of the sphenopalatine artery. Note that '''''these hypervascular masses should not be biopsied''''', as this may result in significant bleeding that is hard to control in the clinic setting.

=== Laboratory Tests ===
Laboratory tests are not commonly useful in the diagnosis of JNA's, but should be performed preoperatively in the case of a planned surgical resection.
* Hemoglobin / Hematocrit
* Platelet level
* Platelet function (if family history of platelet dysfunction)
* PT / INR
* Blood Type and Screen (all patients)
* Blood Type and Cross (patients with advanced stage tumors where a significant blood loss is expected)

=== Imaging ===
[[File:Holman-Miller Sign.png|right|thumb|300 px|Axial CT scan of a JNA showing a right-sided Holman Miller sign]]
CT scan, angiography, and MRI are all useful in the workup of JNA's and have differing benefits.<ref name="Gaillard 2008">Gaillard F, Walizai T, Bell D, et al. Juvenile nasopharyngeal angiofibroma. Reference article, Radiopaedia.org (Accessed on 17 Nov 2024) https://doi.org/10.53347/rID-1541</ref> CT is typically the first imaging modality ordered in these patients, and is helpful for delineating the extent of bony erosion. Angiography demonstrates the extent of the mass well, and is frequently used in pre-operative embolization 24-48 hours before surgical resection. A majority of JNA's are supplied by the external carotid system, either by the maxillary artery (SPA) or the ascending pharyngeal artery.<ref name="Gaillard 2008"></ref> A lesser fraction of tumors are supplied by the internal carotid system (ophthalmic or sphenoidal branches), and this is typically in larger masses with intracranial invasion. MRI can be used to assess for intraorbital or intracranial extension. JNA's are T1 intermediate, T2 heterogenous with flow voids, and enhance with Gadolinium. The following are general characteristics often found in imaging of JNA's:
* Highly vascular nasopharyngeal mass
* Holman-Miller Sign<ref name="Niknejad 2013">Niknejad M, Tatco V, Bell D, et al. Holman-Miller sign (maxillary sinus). Reference article, Radiopaedia.org (Accessed on 17 Nov 2024) https://doi.org/10.53347/rID-21804</ref>: Anterior displacement of the posterior maxillary wall due to mass effect from the tumor
* Widening of the PMF or PPF
* Erosion of the pterygoid plate, especially the medial plate

There are several classification systems that have been proposed for JNAs based on imaging findings.

{| class="wikitable", style="margin-left: auto; margin-right: auto; border: none; text-align: center"
|+ Classification Systems for Juvenile Nasopharyngeal Angiofibromas
|-
! Classification System !! style=width:15em | Stage I !! style=width:15em | Stage II !! style=width:15em | Stage III !! style=width:15em | Stage IV !! style=width:15em | Stage V
|-
| Andrews (1989)<ref name="Andrews 1989">Andrews JC, Fisch U, Aeppli U, Valavanis A, Makek MS. The surgical management of extensive nasopharyngeal angiofibromas with the infratemporal fossa approach. The Laryngoscope. 1989 Apr;99(4):429-37. https://doi.org/10.1288/00005537-198904000-00013</ref> || Confined to NP || Invading one of the following with evidence of bony erosion: PPF, MS, ES, or SS || Invading ITF or orbit '''IIIa''': No intracranial extension '''IIIb''': Extradural (parasellar) extension || Intradural extension '''IVa''': No infiltration of CS, PF, or OC '''IVb''': Infiltration of CS, PF, or OC || --
|-
| Chandler (1984)<ref name="Chandler 1984">Chandler JR, Moskowitz L, Goulding R, Quencer RM. Nasopharyngeal angiofibromas: staging and management. Annals of Otology, Rhinology & Laryngology. 1984 Jul;93(4):322-9. https://doi.org/10.1177/000348948409300408</ref> || Confined to NP || Extension into the nasal cavity, SS, or both || Extension into any of the following: antrum, ES, PMF, ITF, orbit, or cheek || Intracranial extension || --
|-
| Onerci (2006)<ref name="Onerci 2006">Onerci M, Oğretmenoğlu O, Yücel T. Juvenile nasopharyngeal angiofibroma: a revised staging system. Rhinology. 2006 Mar 1;44(1):39-45. PMID: 16550949</ref>|| Nose, NP, ES, and SS or minimal extension into PMF || MS involvement, full occupation of PMF, extension to anterior cranial fossa, limited extension into ITF || Deep extension into cancellous bone at pterygoid base or body and GW of sphenoid, significant lateral extension into ITF or pterygoid plates, orbital involvement, CS obliteration || Intracranial extension between pituitary gland and ICA, tumor localization lateral to ICA, middle fossa extension and extensive intracranial extension || --
|-
| Radkowski (1996)<ref name="Radkowski 1996">Radkowski D, McGill T, Healy GB, Ohlms L, Jones DT. Angiofibroma: changes in staging and treatment. Archives of Otolaryngology–Head & Neck Surgery. 1996 Feb 1;122(2):122-9. https://doi.org/10.1001/archotol.1996.01890140012004</ref>|| '''IA''': Confined to nose or NP '''IB''': Extends into one or more sinuses || '''IIa''': Minimal extension into medial PMF '''IIb''': Full occupation of PMF with local mass effect '''IIc''': Extension into ITF, cheek, or posterior to pterygoid plates || Erosion of skull base '''IIIa''': Minimal skull base involvement '''IIIb''': Extensive intracranial extension, with or without invasion into CS || -- || --
|-
| Sessions (1981)<ref name="Sessions 1981">Sessions RB, Bryan RN, Naclerio RM, Alford BR. Radiographic staging of juvenile angiofibroma. Head & neck surgery. 1981 Mar;3(4):279-83. https://doi.org/10.1002/hed.2890030404</ref> || '''IA''': Confined to nose or NP '''IB''': Extends into one or more sinuses || '''IIa''': Minimal extension into PMF '''IIb''': Full occupation of PMF with or without orbital erosion '''IIc''': ITF with or without cheek extension || Intracranial extension || -- || --
|-
| UPMC (2010)<ref name="Snyderman 2010">Snyderman CH, Pant H, Carrau RL, Gardner P. A new endoscopic staging system for angiofibromas. Archives of Otolaryngology–Head & Neck Surgery. 2010 Jun 21;136(6):588-94. https://doi.org/10.1001/archoto.2010.83</ref> || Nasal cavity, medial PPF || Paranasal sinuses, lateral PPF; no residual vascularity || Skull base erosion, orbit, ITF involvement; no residual vascularity || Skull base erosion, orbit, ITF involvement; residual vascularity || Intracranial extension with residual vascularity '''M''': Medial extension '''L''': Lateral extension
|-
|+ style="caption-side:bottom; font-weight: normal;"|''Abbreviations: Cavernous sinus (CS), Ethmoid sinus (ES), Infratemporal fossa (ITF), Internal carotid artery (ICA), Maxillary sinus (MS), Nasopharynx (NP), Optic chiasm (OC), Pituitary fossa (PF), Pterygomaxillary fissure (PMF), Pterygopalatine fossa (PPF), Sphenoid sinus (SS)''
|}

=== Differential Diagnosis ===
In the case of a nasopharyngeal mass, the following should also be considered:
* Clival chondroma
* Encephalocele
* Esthesioneuroblastoma
* Inverted papilloma
* Nasal polyp
* Nasopharyngeal carcinoma
* Nasopharyngeal teratoma
* Rhabdomyosarcoma
* Vascular malformation

== Management ==
=== Medical Management ===
There is no approved medical treatment for management of juvenile nasopharyngeal angiofibromas. The treatment is primarily surgical. Medical management focuses on optimizing patients before surgery, such as addressing any coagulopathies or addressing underlying anemia.

=== Surgical Management ===
Historically, surgical management of JNAs involved aggressive open approaches, including transpalatal, lateral rhinotomy, midface degloving, or infratemporal fossa craniotomy approaches.<ref name="Renkonen 2011">Renkonen S, Hagström J, Vuola J, Niemelä M, Porras M, Kivivuori SM, Leivo I, Mäkitie AA. The changing surgical management of juvenile nasopharyngeal angiofibroma. European archives of oto-rhino-laryngology. 2011 Apr;268:599-607. https://doi.org/10.1007/s00405-010-1383-z</ref> In recent years, there has been a transition towards transnasal endoscopic approaches. This has resulted in better visualization during surgery and a subsequent improvement in intraoperative blood loss, need for blood transfusions,<ref name="Oliveira 2012">Oliveira JA, Tavares MG, Aguiar CV, Azevedo JF, Sousa JR, Almeida PC, Gomes EF. Comparison between endoscopic and open surgery in 37 patients with nasopharyngeal angiofibroma. Brazilian Journal of otorhinolaryngology. 2012;78:75-80. https://doi.org/10.1590/S1808-86942012000100012</ref> and recurrence rates.<ref name="Bosraty 2011">Bosraty H, Atef A, Aziz M. Endoscopic vs. open surgery for treating large, locally advanced juvenile angiofibromas: A comparison of local control and morbidity outcomes. ENT: Ear, Nose & Throat Journal. 2011 Nov 1;90(11). PMID: 22109921</ref> Prior to surgery, preoperative embolization should be considered to limit blood loss intraoperatively.

== Outcomes ==
=== Complications ===
The primary concerns in the surgical management of juvenile nasopharyngeal angiofibroma are bleeding and recurrence rates. Iatrogenic injuries to structures of the orbit (such as ophthalmoplegia) and skull base (such as facial paresthesias) are also a concern depending on the degree of tumor extension.

==== Bleeding ====
Intraoperative bleeding rates vary based on technique, whether preoperative embolization was used, and tumor staging. Endoscopic surgery is generally accepted to have lower intraoperative blood loss relative to more traditional open approaches.<ref name="Oliveira 2012"></ref> Preoperative embolization has also been found to significantly lower intraoperative blood loss. One study of 47 patients (primarily Radkowski stages I-II) found that patients who underwent preoperative embolization had a mean estimated blood loss (EBL) of 770 mL, and patients who did not undergo embolization had a mean EBL of more than 1,400 mL.<ref name="Ardehali 2010">Ardehali MM, Ardestani SH, Yazdani N, Goodarzi H, Bastaninejad S. Endoscopic approach for excision of juvenile nasopharyngeal angiofibroma: complications and outcomes. American journal of otolaryngology. 2010 Sep 1;31(5):343-9. https://doi.org/10.1016/j.amjoto.2009.04.007</ref> The same study noted that embolized patients had a mean hospital stay of 1.8 days, vs 3.1 days for all patients in the study. A systematic review of advance-stage JNAs with intracranial extension found a mean EBL of 1,700 mL and a similar reduction in EBL following preoperative embolization.<ref name="Leong 2013">Leong SC. A systematic review of surgical outcomes for advanced juvenile nasopharyngeal angiofibroma with intracranial involvement. The Laryngoscope. 2013 May;123(5):1125-31. https://doi.org/10.1002/lary.23760</ref>

==== Recurrence ====

=== Prognosis ===
Recurrence rates can be very high and increase with increased stage at the time of surgery. Recurrence has been reported from 22%<ref name="Bleier 2009">Bleier BS, Kennedy DW, Palmer JN, Chiu AG, Bloom JD, O'Malley Jr BW. Current management of juvenile nasopharyngeal angiofibroma: a tertiary center experience 1999–2007. American journal of rhinology & allergy. 2009 May;23(3):328-30. https://doi.org/10.2500/ajra.2009.23.3322</ref> to as high as 61% in advanced disease.<ref name="Huang 2014">Huang Y, Liu Z, Wang J, Sun X, Yang L, Wang D. Surgical management of juvenile nasopharyngeal angiofibroma: analysis of 162 cases from 1995 to 2012. The Laryngoscope. 2014 Aug;124(8):1942-6. https://doi.org/10.1002/lary.24522</ref>

== References ==
<references />

Juvenile Nasopharyngeal Angiofibroma

2024-11-17T21:47:09Z

Jack.Dewey: /* Prognosis */

{{infobox Disease
|Title =
|Aliases =
|Image = [[File:Nasopharyngeal angiofibroma - 2 - high mag.jpg]]
|Caption = Histologic section of a JNA
|ICD-9 = 210.7
|ICD-10 = D10.6
|MeSH =
|Gene =
|Locus =
|OMIM =
|EyeWiki =
|Radiopaedia = [https://radiopaedia.org/articles/juvenile-nasopharyngeal-angiofibroma?lang=us Juvenile Nasopharyngeal Angiofibroma]
|Pathology = [https://www.pathologyoutlines.com/topic/nasalangiofibroma.html Nasal Angiofibroma]
}}

== Overview ==
'''Juvenile nasopharyngeal angiofibroma (JNA)''' is a benign vascular neoplasm of the nasal cavity and nasopharynx that classically presents in adolescent boys.

== Pathophysiology ==
=== Relevant Anatomy ===
JNA's typically originate in the region of the sphenopalatine artery (SPA) in the posterior nasal cavity/nasopharynx. From there, they can extend into the paranasal sinuses, pterygopalatine fossa (PPF), infratemporal fossa (ITF), orbit, or cranial vault.

<gallery>
Pterygopalatine fossa.jpg|Cadaveric model of the PPF
Schematic diagram showing the bony anatomy and main neural connections of the PPF.png|Neural contents of the PPF
Gray511.png|Maxillary artery branches, with SPA at the distal end
Gerrish's Text-book of Anatomy (1902) - Fig. 243.png|Location of the sphenopalatine foramen
Basilar process and palatine process.jpg|Location of the ITF
</gallery>

=== Disease Etiology ===
JNA's are fibrovascular tumors that arise from the posterior lateral nasal cavity near the choana. There is substantial debate about the exact circumstances and location that lead to the formation of a JNA, but they are generally accepted to form near the region of the sphenopalatine foramen. Proposed theories regarding their etiology include androgen receptor-related growth,<ref name="Liu 2015">Liu Z, Wang J, Wang H, Wang D, Hu L, Liu Q, Sun X. Hormonal receptors and vascular endothelial growth factor in juvenile nasopharyngeal angiofibroma: immunohistochemical and tissue microarray analysis. Acta oto-laryngologica. 2015 Jan 2;135(1):51-7. https://doi.org/10.3109/00016489.2014.952774</ref> incomplete regression of a branchial artery during embryogenesis,<ref name="Schick 2002">Schick B, Plinkert PK, Prescher A. Aetiology of angiofibromas: reflection on their specific vascular component. Laryngo-rhino-otologie. 2002 Apr 1;81(4):280-4. https://doi.org/10.1055/s-2002-25322</ref> oncogenic mutations in C-MYC and C-KIT,<ref name="Pandey 2017">Pandey P, Mishra A, Tripathi AM, Verma V, Trivedi R, Singh HP, Kumar S, Patel B, Singh V, Pandey S, Pandey A. Current molecular profile of juvenile nasopharyngeal angiofibroma: first comprehensive study from India. The Laryngoscope. 2017 Mar;127(3):E100-6. https://doi.org/10.1002/lary.26250</ref> and a wide variety of other genetic aberrations.

===Epidemiology===
Juvenile nasopharyngeal angiofibroma has been reported to be the most common benign neoplasm of the nasopharynx in young males. It accounts for 0.05% to 0.5% of all head and neck tumors, with a reported incidence of 1 in 5,000 to 1 in 60,000 in the US annually.<ref name="Boghani 2013">Boghani Z, Husain Q, Kanumuri VV, Khan MN, Sangvhi S, Liu JK, Eloy JA. Juvenile nasopharyngeal angiofibroma: a systematic review and comparison of endoscopic, endoscopic‐assisted, and open resection in 1047 cases. The Laryngoscope. 2013 Apr;123(4):859-69. https://doi.org/10.1002/lary.23843</ref><ref name="Ferouz 1995">Ferouz AS, Mohr RM, Paul P. Juvenile nasopharyngeal angiofibroma and familial adenomatous polyposis: an association?. Otolaryngology—Head and Neck Surgery. 1995 Oct;113(4):435-9. https://doi.org/10.1016/S0194-59989570081-1</ref><ref name="Huang 2014">Huang Y, Liu Z, Wang J, Sun X, Yang L, Wang D. Surgical management of juvenile nasopharyngeal angiofibroma: analysis of 162 cases from 1995 to 2012. The Laryngoscope. 2014 Aug;124(8):1942-6. https://doi.org/10.1002/lary.24522</ref> It classically presents in adolescent males, with a typical age of presentation between 13 and 22 years old.<ref name ="Newman 2023">Newman M, Nguyen TB, McHugh T, Reddy K, Sommer DD. Early-onset juvenile nasopharyngeal angiofibroma (JNA): a systematic review. Journal of Otolaryngology-Head & Neck Surgery. 2023 Dec;52(1):s40463-023. https://doi.org/10.1186/s40463-023-00687-w</ref>

=== Genetics ===
There is no identified definitive genetic locus that predisposes patients to JNA's. However, there have been a number of studies looking at the various genetic components that could influence their formation.<ref name="Doody 2019">Doody J, Adil EA, Trenor III CC, Cunningham MJ. The genetic and molecular determinants of juvenile nasopharyngeal angiofibroma: a systematic review. Annals of Otology, Rhinology & Laryngology. 2019 Nov;128(11):1061-72. https://doi.org/10.1177/0003489419850194</ref> Due to fact that JNA's classically form in adolescent males, there have been several studies focused on the possible hormonal influence on their formation and growth. JNA's tend to have an abundance of progesterone and estradiol, but a relatively lower concentration of testosterone and dihydrotestosterone.<ref name="Kumagami 05 1993">Kumagami H. Estradiol, dihydrotestosterone, and testosterone in juvenile nasopharyngeal angiofibroma tissue. American Journal of Rhinology. 1993 May;7(3):101-4. https://doi.org/10.2500/105065893781976393</ref><ref name="Kumagami 01 1993">Kumagami H. Sex hormones in juvenile nasopharyngeal angiofibroma tissue. Auris Nasus Larynx. 1993 Jan 1;20(2):131-5. https://doi.org/10.1016/S0385-8146(12)80240-9</ref> Supplemental testosterone was trialed in JNA patients, but both tumor growth<ref name="Johnsen 1966">Johnsen S, Kloster JH, Schiff M. The action of hormones on juvenile nasopharyngeal angiofibroma. Acta Oto-Laryngologica. 1966 Jan 1;61(1-6):153-60. https://doi.org/10.3109/00016486609127052</ref> and tumor recurrence<ref name="Riggs 2010">Riggs S, Orlandi RR. Juvenile nasopharyngeal angiofibroma recurrence associated with exogenous testosterone therapy. Head & Neck: Journal for the Sciences and Specialties of the Head and Neck. 2010 Jun;32(6):812-5. https://doi.org/10.1002/hed.21152</ref> have been documented. Papers investigating potential targeted therapy markers have looked at steroid hormones, chromosomal abnormalities, growth factors, and other intracellular molecular targets.<ref name="Doody 2019"></ref> There have been studies investigating a possible link between Familial Adenomatous Polyposis (FAP) and JNA's via the adenomatous polyposis coli (APC)/β-catenin pathway, but no specific genetic link has been identified as of yet.<ref name="Guertl 2000">Guertl B, Beham A, Zechner R, Stammberger H, Hoefler G. Nasopharyngeal angiofibroma: an AM-Gene-Associated tumor?. Human pathology. 2000 Nov 1;31(11):1411-3.</ref>

=== Histology ===
Grossly on histologic section there are large fibrous/collagenous sections with fibroblasts, as well as vascular spaces of various sizes throughout the tumor.<ref name="Xu 2020">Xu B. Nasopharyngeal angiofibroma. PathologyOutlines.com website. https://www.pathologyoutlines.com/topic/nasalangiofibroma.html. Accessed November 17th, 2024.</ref> The stromal cells have an abundance of beta-catenin relative to the vascular endothelial cells.

<gallery>
JNA 30x.png|H&E stained section of green inked vascular channel (30x)
Beta Catenin JNA.png|Abnormal nuclear expression in stromal cells (blue arrow), compared with membranous / cytoplasmic staining in the endothelial cells (red arrow)
</gallery>

== Diagnosis ==
=== Patient History ===
Patients may have a history of any of the following symptoms:
* Unilateral nasal obstruction
* Recurrent epistaxis
* High-volume epistaxis
* Eustachian tube dysfunction
* Headache
* Facial swelling
* Anosmia / Hyposmia
* Cranial neuropathy
* Vision changes

=== Physical Examination ===
Many patients will not have obvious findings on anterior rhinoscopy. A large vascular-appearing mass is commonly fond on nasal endoscopy on the posterior lateral nasal sidewall near the region of the sphenopalatine artery. Note that '''''these hypervascular masses should not be biopsied''''', as this may result in significant bleeding that is hard to control in the clinic setting.

=== Laboratory Tests ===
Laboratory tests are not commonly useful in the diagnosis of JNA's, but should be performed preoperatively in the case of a planned surgical resection.
* Hemoglobin / Hematocrit
* Platelet level
* Platelet function (if family history of platelet dysfunction)
* PT / INR
* Blood Type and Screen (all patients)
* Blood Type and Cross (patients with advanced stage tumors where a significant blood loss is expected)

=== Imaging ===
[[File:Holman-Miller Sign.png|right|thumb|300 px|Axial CT scan of a JNA showing a right-sided Holman Miller sign]]
CT scan, angiography, and MRI are all useful in the workup of JNA's and have differing benefits.<ref name="Gaillard 2008">Gaillard F, Walizai T, Bell D, et al. Juvenile nasopharyngeal angiofibroma. Reference article, Radiopaedia.org (Accessed on 17 Nov 2024) https://doi.org/10.53347/rID-1541</ref> CT is typically the first imaging modality ordered in these patients, and is helpful for delineating the extent of bony erosion. Angiography demonstrates the extent of the mass well, and is frequently used in pre-operative embolization 24-48 hours before surgical resection. A majority of JNA's are supplied by the external carotid system, either by the maxillary artery (SPA) or the ascending pharyngeal artery.<ref name="Gaillard 2008"></ref> A lesser fraction of tumors are supplied by the internal carotid system (ophthalmic or sphenoidal branches), and this is typically in larger masses with intracranial invasion. MRI can be used to assess for intraorbital or intracranial extension. JNA's are T1 intermediate, T2 heterogenous with flow voids, and enhance with Gadolinium. The following are general characteristics often found in imaging of JNA's:
* Highly vascular nasopharyngeal mass
* Holman-Miller Sign<ref name="Niknejad 2013">Niknejad M, Tatco V, Bell D, et al. Holman-Miller sign (maxillary sinus). Reference article, Radiopaedia.org (Accessed on 17 Nov 2024) https://doi.org/10.53347/rID-21804</ref>: Anterior displacement of the posterior maxillary wall due to mass effect from the tumor
* Widening of the PMF or PPF
* Erosion of the pterygoid plate, especially the medial plate

There are several classification systems that have been proposed for JNAs based on imaging findings.

{| class="wikitable", style="margin-left: auto; margin-right: auto; border: none; text-align: center"
|+ Classification Systems for Juvenile Nasopharyngeal Angiofibromas
|-
! Classification System !! style=width:15em | Stage I !! style=width:15em | Stage II !! style=width:15em | Stage III !! style=width:15em | Stage IV !! style=width:15em | Stage V
|-
| Andrews (1989)<ref name="Andrews 1989">Andrews JC, Fisch U, Aeppli U, Valavanis A, Makek MS. The surgical management of extensive nasopharyngeal angiofibromas with the infratemporal fossa approach. The Laryngoscope. 1989 Apr;99(4):429-37. https://doi.org/10.1288/00005537-198904000-00013</ref> || Confined to NP || Invading one of the following with evidence of bony erosion: PPF, MS, ES, or SS || Invading ITF or orbit '''IIIa''': No intracranial extension '''IIIb''': Extradural (parasellar) extension || Intradural extension '''IVa''': No infiltration of CS, PF, or OC '''IVb''': Infiltration of CS, PF, or OC || --
|-
| Chandler (1984)<ref name="Chandler 1984">Chandler JR, Moskowitz L, Goulding R, Quencer RM. Nasopharyngeal angiofibromas: staging and management. Annals of Otology, Rhinology & Laryngology. 1984 Jul;93(4):322-9. https://doi.org/10.1177/000348948409300408</ref> || Confined to NP || Extension into the nasal cavity, SS, or both || Extension into any of the following: antrum, ES, PMF, ITF, orbit, or cheek || Intracranial extension || --
|-
| Onerci (2006)<ref name="Onerci 2006">Onerci M, Oğretmenoğlu O, Yücel T. Juvenile nasopharyngeal angiofibroma: a revised staging system. Rhinology. 2006 Mar 1;44(1):39-45. PMID: 16550949</ref>|| Nose, NP, ES, and SS or minimal extension into PMF || MS involvement, full occupation of PMF, extension to anterior cranial fossa, limited extension into ITF || Deep extension into cancellous bone at pterygoid base or body and GW of sphenoid, significant lateral extension into ITF or pterygoid plates, orbital involvement, CS obliteration || Intracranial extension between pituitary gland and ICA, tumor localization lateral to ICA, middle fossa extension and extensive intracranial extension || --
|-
| Radkowski (1996)<ref name="Radkowski 1996">Radkowski D, McGill T, Healy GB, Ohlms L, Jones DT. Angiofibroma: changes in staging and treatment. Archives of Otolaryngology–Head & Neck Surgery. 1996 Feb 1;122(2):122-9. https://doi.org/10.1001/archotol.1996.01890140012004</ref>|| '''IA''': Confined to nose or NP '''IB''': Extends into one or more sinuses || '''IIa''': Minimal extension into medial PMF '''IIb''': Full occupation of PMF with local mass effect '''IIc''': Extension into ITF, cheek, or posterior to pterygoid plates || Erosion of skull base '''IIIa''': Minimal skull base involvement '''IIIb''': Extensive intracranial extension, with or without invasion into CS || -- || --
|-
| Sessions (1981)<ref name="Sessions 1981">Sessions RB, Bryan RN, Naclerio RM, Alford BR. Radiographic staging of juvenile angiofibroma. Head & neck surgery. 1981 Mar;3(4):279-83. https://doi.org/10.1002/hed.2890030404</ref> || '''IA''': Confined to nose or NP '''IB''': Extends into one or more sinuses || '''IIa''': Minimal extension into PMF '''IIb''': Full occupation of PMF with or without orbital erosion '''IIc''': ITF with or without cheek extension || Intracranial extension || -- || --
|-
| UPMC (2010)<ref name="Snyderman 2010">Snyderman CH, Pant H, Carrau RL, Gardner P. A new endoscopic staging system for angiofibromas. Archives of Otolaryngology–Head & Neck Surgery. 2010 Jun 21;136(6):588-94. https://doi.org/10.1001/archoto.2010.83</ref> || Nasal cavity, medial PPF || Paranasal sinuses, lateral PPF; no residual vascularity || Skull base erosion, orbit, ITF involvement; no residual vascularity || Skull base erosion, orbit, ITF involvement; residual vascularity || Intracranial extension with residual vascularity '''M''': Medial extension '''L''': Lateral extension
|-
|+ style="caption-side:bottom; font-weight: normal;"|''Abbreviations: Cavernous sinus (CS), Ethmoid sinus (ES), Infratemporal fossa (ITF), Internal carotid artery (ICA), Maxillary sinus (MS), Nasopharynx (NP), Optic chiasm (OC), Pituitary fossa (PF), Pterygomaxillary fissure (PMF), Pterygopalatine fossa (PPF), Sphenoid sinus (SS)''
|}

=== Differential Diagnosis ===
In the case of a nasopharyngeal mass, the following should also be considered:
* Clival chondroma
* Encephalocele
* Esthesioneuroblastoma
* Inverted papilloma
* Nasal polyp
* Nasopharyngeal carcinoma
* Nasopharyngeal teratoma
* Rhabdomyosarcoma
* Vascular malformation

== Management ==
=== Medical Management ===
There is no approved medical treatment for management of juvenile nasopharyngeal angiofibromas. The treatment is primarily surgical. Medical management focuses on optimizing patients before surgery, such as addressing any coagulopathies or addressing underlying anemia.

=== Surgical Management ===
Historically, surgical management of JNAs involved aggressive open approaches, including transpalatal, lateral rhinotomy, midface degloving, or infratemporal fossa craniotomy approaches.<ref name="Renkonen 2011">Renkonen S, Hagström J, Vuola J, Niemelä M, Porras M, Kivivuori SM, Leivo I, Mäkitie AA. The changing surgical management of juvenile nasopharyngeal angiofibroma. European archives of oto-rhino-laryngology. 2011 Apr;268:599-607. https://doi.org/10.1007/s00405-010-1383-z</ref> In recent years, there has been a transition towards transnasal endoscopic approaches. This has resulted in better visualization during surgery and a subsequent improvement in intraoperative blood loss, need for blood transfusions,<ref name="Oliveira 2012">Oliveira JA, Tavares MG, Aguiar CV, Azevedo JF, Sousa JR, Almeida PC, Gomes EF. Comparison between endoscopic and open surgery in 37 patients with nasopharyngeal angiofibroma. Brazilian Journal of otorhinolaryngology. 2012;78:75-80. https://doi.org/10.1590/S1808-86942012000100012</ref> and recurrence rates.<ref name="Bosraty 2011">Bosraty H, Atef A, Aziz M. Endoscopic vs. open surgery for treating large, locally advanced juvenile angiofibromas: A comparison of local control and morbidity outcomes. ENT: Ear, Nose & Throat Journal. 2011 Nov 1;90(11). PMID: 22109921</ref> Prior to surgery, preoperative embolization should be considered to limit blood loss intraoperatively.

== Outcomes ==
=== Complications ===
=== Prognosis ===
Recurrence rates can be very high and increase with increased stage at the time of surgery. Recurrence has been reported from 22%<ref name="Bleier 2009">Bleier BS, Kennedy DW, Palmer JN, Chiu AG, Bloom JD, O'Malley Jr BW. Current management of juvenile nasopharyngeal angiofibroma: a tertiary center experience 1999–2007. American journal of rhinology & allergy. 2009 May;23(3):328-30. https://doi.org/10.2500/ajra.2009.23.3322</ref> to as high as 61% in advanced disease.<ref name="Huang 2014">Huang Y, Liu Z, Wang J, Sun X, Yang L, Wang D. Surgical management of juvenile nasopharyngeal angiofibroma: analysis of 162 cases from 1995 to 2012. The Laryngoscope. 2014 Aug;124(8):1942-6. https://doi.org/10.1002/lary.24522</ref>

== References ==
<references />

Juvenile Nasopharyngeal Angiofibroma

2024-11-17T21:46:07Z

Jack.Dewey: /* Prognosis */

{{infobox Disease
|Title =
|Aliases =
|Image = [[File:Nasopharyngeal angiofibroma - 2 - high mag.jpg]]
|Caption = Histologic section of a JNA
|ICD-9 = 210.7
|ICD-10 = D10.6
|MeSH =
|Gene =
|Locus =
|OMIM =
|EyeWiki =
|Radiopaedia = [https://radiopaedia.org/articles/juvenile-nasopharyngeal-angiofibroma?lang=us Juvenile Nasopharyngeal Angiofibroma]
|Pathology = [https://www.pathologyoutlines.com/topic/nasalangiofibroma.html Nasal Angiofibroma]
}}

== Overview ==
'''Juvenile nasopharyngeal angiofibroma (JNA)''' is a benign vascular neoplasm of the nasal cavity and nasopharynx that classically presents in adolescent boys.

== Pathophysiology ==
=== Relevant Anatomy ===
JNA's typically originate in the region of the sphenopalatine artery (SPA) in the posterior nasal cavity/nasopharynx. From there, they can extend into the paranasal sinuses, pterygopalatine fossa (PPF), infratemporal fossa (ITF), orbit, or cranial vault.

<gallery>
Pterygopalatine fossa.jpg|Cadaveric model of the PPF
Schematic diagram showing the bony anatomy and main neural connections of the PPF.png|Neural contents of the PPF
Gray511.png|Maxillary artery branches, with SPA at the distal end
Gerrish's Text-book of Anatomy (1902) - Fig. 243.png|Location of the sphenopalatine foramen
Basilar process and palatine process.jpg|Location of the ITF
</gallery>

=== Disease Etiology ===
JNA's are fibrovascular tumors that arise from the posterior lateral nasal cavity near the choana. There is substantial debate about the exact circumstances and location that lead to the formation of a JNA, but they are generally accepted to form near the region of the sphenopalatine foramen. Proposed theories regarding their etiology include androgen receptor-related growth,<ref name="Liu 2015">Liu Z, Wang J, Wang H, Wang D, Hu L, Liu Q, Sun X. Hormonal receptors and vascular endothelial growth factor in juvenile nasopharyngeal angiofibroma: immunohistochemical and tissue microarray analysis. Acta oto-laryngologica. 2015 Jan 2;135(1):51-7. https://doi.org/10.3109/00016489.2014.952774</ref> incomplete regression of a branchial artery during embryogenesis,<ref name="Schick 2002">Schick B, Plinkert PK, Prescher A. Aetiology of angiofibromas: reflection on their specific vascular component. Laryngo-rhino-otologie. 2002 Apr 1;81(4):280-4. https://doi.org/10.1055/s-2002-25322</ref> oncogenic mutations in C-MYC and C-KIT,<ref name="Pandey 2017">Pandey P, Mishra A, Tripathi AM, Verma V, Trivedi R, Singh HP, Kumar S, Patel B, Singh V, Pandey S, Pandey A. Current molecular profile of juvenile nasopharyngeal angiofibroma: first comprehensive study from India. The Laryngoscope. 2017 Mar;127(3):E100-6. https://doi.org/10.1002/lary.26250</ref> and a wide variety of other genetic aberrations.

===Epidemiology===
Juvenile nasopharyngeal angiofibroma has been reported to be the most common benign neoplasm of the nasopharynx in young males. It accounts for 0.05% to 0.5% of all head and neck tumors, with a reported incidence of 1 in 5,000 to 1 in 60,000 in the US annually.<ref name="Boghani 2013">Boghani Z, Husain Q, Kanumuri VV, Khan MN, Sangvhi S, Liu JK, Eloy JA. Juvenile nasopharyngeal angiofibroma: a systematic review and comparison of endoscopic, endoscopic‐assisted, and open resection in 1047 cases. The Laryngoscope. 2013 Apr;123(4):859-69. https://doi.org/10.1002/lary.23843</ref><ref name="Ferouz 1995">Ferouz AS, Mohr RM, Paul P. Juvenile nasopharyngeal angiofibroma and familial adenomatous polyposis: an association?. Otolaryngology—Head and Neck Surgery. 1995 Oct;113(4):435-9. https://doi.org/10.1016/S0194-59989570081-1</ref><ref name="Huang 2014">Huang Y, Liu Z, Wang J, Sun X, Yang L, Wang D. Surgical management of juvenile nasopharyngeal angiofibroma: analysis of 162 cases from 1995 to 2012. The Laryngoscope. 2014 Aug;124(8):1942-6. https://doi.org/10.1002/lary.24522</ref> It classically presents in adolescent males, with a typical age of presentation between 13 and 22 years old.<ref name ="Newman 2023">Newman M, Nguyen TB, McHugh T, Reddy K, Sommer DD. Early-onset juvenile nasopharyngeal angiofibroma (JNA): a systematic review. Journal of Otolaryngology-Head & Neck Surgery. 2023 Dec;52(1):s40463-023. https://doi.org/10.1186/s40463-023-00687-w</ref>

=== Genetics ===
There is no identified definitive genetic locus that predisposes patients to JNA's. However, there have been a number of studies looking at the various genetic components that could influence their formation.<ref name="Doody 2019">Doody J, Adil EA, Trenor III CC, Cunningham MJ. The genetic and molecular determinants of juvenile nasopharyngeal angiofibroma: a systematic review. Annals of Otology, Rhinology & Laryngology. 2019 Nov;128(11):1061-72. https://doi.org/10.1177/0003489419850194</ref> Due to fact that JNA's classically form in adolescent males, there have been several studies focused on the possible hormonal influence on their formation and growth. JNA's tend to have an abundance of progesterone and estradiol, but a relatively lower concentration of testosterone and dihydrotestosterone.<ref name="Kumagami 05 1993">Kumagami H. Estradiol, dihydrotestosterone, and testosterone in juvenile nasopharyngeal angiofibroma tissue. American Journal of Rhinology. 1993 May;7(3):101-4. https://doi.org/10.2500/105065893781976393</ref><ref name="Kumagami 01 1993">Kumagami H. Sex hormones in juvenile nasopharyngeal angiofibroma tissue. Auris Nasus Larynx. 1993 Jan 1;20(2):131-5. https://doi.org/10.1016/S0385-8146(12)80240-9</ref> Supplemental testosterone was trialed in JNA patients, but both tumor growth<ref name="Johnsen 1966">Johnsen S, Kloster JH, Schiff M. The action of hormones on juvenile nasopharyngeal angiofibroma. Acta Oto-Laryngologica. 1966 Jan 1;61(1-6):153-60. https://doi.org/10.3109/00016486609127052</ref> and tumor recurrence<ref name="Riggs 2010">Riggs S, Orlandi RR. Juvenile nasopharyngeal angiofibroma recurrence associated with exogenous testosterone therapy. Head & Neck: Journal for the Sciences and Specialties of the Head and Neck. 2010 Jun;32(6):812-5. https://doi.org/10.1002/hed.21152</ref> have been documented. Papers investigating potential targeted therapy markers have looked at steroid hormones, chromosomal abnormalities, growth factors, and other intracellular molecular targets.<ref name="Doody 2019"></ref> There have been studies investigating a possible link between Familial Adenomatous Polyposis (FAP) and JNA's via the adenomatous polyposis coli (APC)/β-catenin pathway, but no specific genetic link has been identified as of yet.<ref name="Guertl 2000">Guertl B, Beham A, Zechner R, Stammberger H, Hoefler G. Nasopharyngeal angiofibroma: an AM-Gene-Associated tumor?. Human pathology. 2000 Nov 1;31(11):1411-3.</ref>

=== Histology ===
Grossly on histologic section there are large fibrous/collagenous sections with fibroblasts, as well as vascular spaces of various sizes throughout the tumor.<ref name="Xu 2020">Xu B. Nasopharyngeal angiofibroma. PathologyOutlines.com website. https://www.pathologyoutlines.com/topic/nasalangiofibroma.html. Accessed November 17th, 2024.</ref> The stromal cells have an abundance of beta-catenin relative to the vascular endothelial cells.

<gallery>
JNA 30x.png|H&E stained section of green inked vascular channel (30x)
Beta Catenin JNA.png|Abnormal nuclear expression in stromal cells (blue arrow), compared with membranous / cytoplasmic staining in the endothelial cells (red arrow)
</gallery>

== Diagnosis ==
=== Patient History ===
Patients may have a history of any of the following symptoms:
* Unilateral nasal obstruction
* Recurrent epistaxis
* High-volume epistaxis
* Eustachian tube dysfunction
* Headache
* Facial swelling
* Anosmia / Hyposmia
* Cranial neuropathy
* Vision changes

=== Physical Examination ===
Many patients will not have obvious findings on anterior rhinoscopy. A large vascular-appearing mass is commonly fond on nasal endoscopy on the posterior lateral nasal sidewall near the region of the sphenopalatine artery. Note that '''''these hypervascular masses should not be biopsied''''', as this may result in significant bleeding that is hard to control in the clinic setting.

=== Laboratory Tests ===
Laboratory tests are not commonly useful in the diagnosis of JNA's, but should be performed preoperatively in the case of a planned surgical resection.
* Hemoglobin / Hematocrit
* Platelet level
* Platelet function (if family history of platelet dysfunction)
* PT / INR
* Blood Type and Screen (all patients)
* Blood Type and Cross (patients with advanced stage tumors where a significant blood loss is expected)

=== Imaging ===
[[File:Holman-Miller Sign.png|right|thumb|300 px|Axial CT scan of a JNA showing a right-sided Holman Miller sign]]
CT scan, angiography, and MRI are all useful in the workup of JNA's and have differing benefits.<ref name="Gaillard 2008">Gaillard F, Walizai T, Bell D, et al. Juvenile nasopharyngeal angiofibroma. Reference article, Radiopaedia.org (Accessed on 17 Nov 2024) https://doi.org/10.53347/rID-1541</ref> CT is typically the first imaging modality ordered in these patients, and is helpful for delineating the extent of bony erosion. Angiography demonstrates the extent of the mass well, and is frequently used in pre-operative embolization 24-48 hours before surgical resection. A majority of JNA's are supplied by the external carotid system, either by the maxillary artery (SPA) or the ascending pharyngeal artery.<ref name="Gaillard 2008"></ref> A lesser fraction of tumors are supplied by the internal carotid system (ophthalmic or sphenoidal branches), and this is typically in larger masses with intracranial invasion. MRI can be used to assess for intraorbital or intracranial extension. JNA's are T1 intermediate, T2 heterogenous with flow voids, and enhance with Gadolinium. The following are general characteristics often found in imaging of JNA's:
* Highly vascular nasopharyngeal mass
* Holman-Miller Sign<ref name="Niknejad 2013">Niknejad M, Tatco V, Bell D, et al. Holman-Miller sign (maxillary sinus). Reference article, Radiopaedia.org (Accessed on 17 Nov 2024) https://doi.org/10.53347/rID-21804</ref>: Anterior displacement of the posterior maxillary wall due to mass effect from the tumor
* Widening of the PMF or PPF
* Erosion of the pterygoid plate, especially the medial plate

There are several classification systems that have been proposed for JNAs based on imaging findings.

{| class="wikitable", style="margin-left: auto; margin-right: auto; border: none; text-align: center"
|+ Classification Systems for Juvenile Nasopharyngeal Angiofibromas
|-
! Classification System !! style=width:15em | Stage I !! style=width:15em | Stage II !! style=width:15em | Stage III !! style=width:15em | Stage IV !! style=width:15em | Stage V
|-
| Andrews (1989)<ref name="Andrews 1989">Andrews JC, Fisch U, Aeppli U, Valavanis A, Makek MS. The surgical management of extensive nasopharyngeal angiofibromas with the infratemporal fossa approach. The Laryngoscope. 1989 Apr;99(4):429-37. https://doi.org/10.1288/00005537-198904000-00013</ref> || Confined to NP || Invading one of the following with evidence of bony erosion: PPF, MS, ES, or SS || Invading ITF or orbit '''IIIa''': No intracranial extension '''IIIb''': Extradural (parasellar) extension || Intradural extension '''IVa''': No infiltration of CS, PF, or OC '''IVb''': Infiltration of CS, PF, or OC || --
|-
| Chandler (1984)<ref name="Chandler 1984">Chandler JR, Moskowitz L, Goulding R, Quencer RM. Nasopharyngeal angiofibromas: staging and management. Annals of Otology, Rhinology & Laryngology. 1984 Jul;93(4):322-9. https://doi.org/10.1177/000348948409300408</ref> || Confined to NP || Extension into the nasal cavity, SS, or both || Extension into any of the following: antrum, ES, PMF, ITF, orbit, or cheek || Intracranial extension || --
|-
| Onerci (2006)<ref name="Onerci 2006">Onerci M, Oğretmenoğlu O, Yücel T. Juvenile nasopharyngeal angiofibroma: a revised staging system. Rhinology. 2006 Mar 1;44(1):39-45. PMID: 16550949</ref>|| Nose, NP, ES, and SS or minimal extension into PMF || MS involvement, full occupation of PMF, extension to anterior cranial fossa, limited extension into ITF || Deep extension into cancellous bone at pterygoid base or body and GW of sphenoid, significant lateral extension into ITF or pterygoid plates, orbital involvement, CS obliteration || Intracranial extension between pituitary gland and ICA, tumor localization lateral to ICA, middle fossa extension and extensive intracranial extension || --
|-
| Radkowski (1996)<ref name="Radkowski 1996">Radkowski D, McGill T, Healy GB, Ohlms L, Jones DT. Angiofibroma: changes in staging and treatment. Archives of Otolaryngology–Head & Neck Surgery. 1996 Feb 1;122(2):122-9. https://doi.org/10.1001/archotol.1996.01890140012004</ref>|| '''IA''': Confined to nose or NP '''IB''': Extends into one or more sinuses || '''IIa''': Minimal extension into medial PMF '''IIb''': Full occupation of PMF with local mass effect '''IIc''': Extension into ITF, cheek, or posterior to pterygoid plates || Erosion of skull base '''IIIa''': Minimal skull base involvement '''IIIb''': Extensive intracranial extension, with or without invasion into CS || -- || --
|-
| Sessions (1981)<ref name="Sessions 1981">Sessions RB, Bryan RN, Naclerio RM, Alford BR. Radiographic staging of juvenile angiofibroma. Head & neck surgery. 1981 Mar;3(4):279-83. https://doi.org/10.1002/hed.2890030404</ref> || '''IA''': Confined to nose or NP '''IB''': Extends into one or more sinuses || '''IIa''': Minimal extension into PMF '''IIb''': Full occupation of PMF with or without orbital erosion '''IIc''': ITF with or without cheek extension || Intracranial extension || -- || --
|-
| UPMC (2010)<ref name="Snyderman 2010">Snyderman CH, Pant H, Carrau RL, Gardner P. A new endoscopic staging system for angiofibromas. Archives of Otolaryngology–Head & Neck Surgery. 2010 Jun 21;136(6):588-94. https://doi.org/10.1001/archoto.2010.83</ref> || Nasal cavity, medial PPF || Paranasal sinuses, lateral PPF; no residual vascularity || Skull base erosion, orbit, ITF involvement; no residual vascularity || Skull base erosion, orbit, ITF involvement; residual vascularity || Intracranial extension with residual vascularity '''M''': Medial extension '''L''': Lateral extension
|-
|+ style="caption-side:bottom; font-weight: normal;"|''Abbreviations: Cavernous sinus (CS), Ethmoid sinus (ES), Infratemporal fossa (ITF), Internal carotid artery (ICA), Maxillary sinus (MS), Nasopharynx (NP), Optic chiasm (OC), Pituitary fossa (PF), Pterygomaxillary fissure (PMF), Pterygopalatine fossa (PPF), Sphenoid sinus (SS)''
|}

=== Differential Diagnosis ===
In the case of a nasopharyngeal mass, the following should also be considered:
* Clival chondroma
* Encephalocele
* Esthesioneuroblastoma
* Inverted papilloma
* Nasal polyp
* Nasopharyngeal carcinoma
* Nasopharyngeal teratoma
* Rhabdomyosarcoma
* Vascular malformation

== Management ==
=== Medical Management ===
There is no approved medical treatment for management of juvenile nasopharyngeal angiofibromas. The treatment is primarily surgical. Medical management focuses on optimizing patients before surgery, such as addressing any coagulopathies or addressing underlying anemia.

=== Surgical Management ===
Historically, surgical management of JNAs involved aggressive open approaches, including transpalatal, lateral rhinotomy, midface degloving, or infratemporal fossa craniotomy approaches.<ref name="Renkonen 2011">Renkonen S, Hagström J, Vuola J, Niemelä M, Porras M, Kivivuori SM, Leivo I, Mäkitie AA. The changing surgical management of juvenile nasopharyngeal angiofibroma. European archives of oto-rhino-laryngology. 2011 Apr;268:599-607. https://doi.org/10.1007/s00405-010-1383-z</ref> In recent years, there has been a transition towards transnasal endoscopic approaches. This has resulted in better visualization during surgery and a subsequent improvement in intraoperative blood loss, need for blood transfusions,<ref name="Oliveira 2012">Oliveira JA, Tavares MG, Aguiar CV, Azevedo JF, Sousa JR, Almeida PC, Gomes EF. Comparison between endoscopic and open surgery in 37 patients with nasopharyngeal angiofibroma. Brazilian Journal of otorhinolaryngology. 2012;78:75-80. https://doi.org/10.1590/S1808-86942012000100012</ref> and recurrence rates.<ref name="Bosraty 2011">Bosraty H, Atef A, Aziz M. Endoscopic vs. open surgery for treating large, locally advanced juvenile angiofibromas: A comparison of local control and morbidity outcomes. ENT: Ear, Nose & Throat Journal. 2011 Nov 1;90(11). PMID: 22109921</ref> Prior to surgery, preoperative embolization should be considered to limit blood loss intraoperatively.

== Outcomes ==
=== Complications ===
=== Prognosis ===
Recurrence rates can be very high and increase with increased stage at the time of surgery. Recurrence has been reported from 22%<ref name="Bleier 2009">Bleier BS, Kennedy DW, Palmer JN, Chiu AG, Bloom JD, O'Malley Jr BW. Current management of juvenile nasopharyngeal angiofibroma: a tertiary center experience 1999–2007. American journal of rhinology & allergy. 2009 May;23(3):328-30. https://doi.org/10.2500/ajra.2009.23.3322</ref> to as high as 61% in advanced disease.<ref name="Huang 2013"></ref>

== References ==
<references />

Juvenile Nasopharyngeal Angiofibroma

2024-11-17T21:43:31Z

Jack.Dewey: /* Prognosis */

{{infobox Disease
|Title =
|Aliases =
|Image = [[File:Nasopharyngeal angiofibroma - 2 - high mag.jpg]]
|Caption = Histologic section of a JNA
|ICD-9 = 210.7
|ICD-10 = D10.6
|MeSH =
|Gene =
|Locus =
|OMIM =
|EyeWiki =
|Radiopaedia = [https://radiopaedia.org/articles/juvenile-nasopharyngeal-angiofibroma?lang=us Juvenile Nasopharyngeal Angiofibroma]
|Pathology = [https://www.pathologyoutlines.com/topic/nasalangiofibroma.html Nasal Angiofibroma]
}}

== Overview ==
'''Juvenile nasopharyngeal angiofibroma (JNA)''' is a benign vascular neoplasm of the nasal cavity and nasopharynx that classically presents in adolescent boys.

== Pathophysiology ==
=== Relevant Anatomy ===
JNA's typically originate in the region of the sphenopalatine artery (SPA) in the posterior nasal cavity/nasopharynx. From there, they can extend into the paranasal sinuses, pterygopalatine fossa (PPF), infratemporal fossa (ITF), orbit, or cranial vault.

<gallery>
Pterygopalatine fossa.jpg|Cadaveric model of the PPF
Schematic diagram showing the bony anatomy and main neural connections of the PPF.png|Neural contents of the PPF
Gray511.png|Maxillary artery branches, with SPA at the distal end
Gerrish's Text-book of Anatomy (1902) - Fig. 243.png|Location of the sphenopalatine foramen
Basilar process and palatine process.jpg|Location of the ITF
</gallery>

=== Disease Etiology ===
JNA's are fibrovascular tumors that arise from the posterior lateral nasal cavity near the choana. There is substantial debate about the exact circumstances and location that lead to the formation of a JNA, but they are generally accepted to form near the region of the sphenopalatine foramen. Proposed theories regarding their etiology include androgen receptor-related growth,<ref name="Liu 2015">Liu Z, Wang J, Wang H, Wang D, Hu L, Liu Q, Sun X. Hormonal receptors and vascular endothelial growth factor in juvenile nasopharyngeal angiofibroma: immunohistochemical and tissue microarray analysis. Acta oto-laryngologica. 2015 Jan 2;135(1):51-7. https://doi.org/10.3109/00016489.2014.952774</ref> incomplete regression of a branchial artery during embryogenesis,<ref name="Schick 2002">Schick B, Plinkert PK, Prescher A. Aetiology of angiofibromas: reflection on their specific vascular component. Laryngo-rhino-otologie. 2002 Apr 1;81(4):280-4. https://doi.org/10.1055/s-2002-25322</ref> oncogenic mutations in C-MYC and C-KIT,<ref name="Pandey 2017">Pandey P, Mishra A, Tripathi AM, Verma V, Trivedi R, Singh HP, Kumar S, Patel B, Singh V, Pandey S, Pandey A. Current molecular profile of juvenile nasopharyngeal angiofibroma: first comprehensive study from India. The Laryngoscope. 2017 Mar;127(3):E100-6. https://doi.org/10.1002/lary.26250</ref> and a wide variety of other genetic aberrations.

===Epidemiology===
Juvenile nasopharyngeal angiofibroma has been reported to be the most common benign neoplasm of the nasopharynx in young males. It accounts for 0.05% to 0.5% of all head and neck tumors, with a reported incidence of 1 in 5,000 to 1 in 60,000 in the US annually.<ref name="Boghani 2013">Boghani Z, Husain Q, Kanumuri VV, Khan MN, Sangvhi S, Liu JK, Eloy JA. Juvenile nasopharyngeal angiofibroma: a systematic review and comparison of endoscopic, endoscopic‐assisted, and open resection in 1047 cases. The Laryngoscope. 2013 Apr;123(4):859-69. https://doi.org/10.1002/lary.23843</ref><ref name="Ferouz 1995">Ferouz AS, Mohr RM, Paul P. Juvenile nasopharyngeal angiofibroma and familial adenomatous polyposis: an association?. Otolaryngology—Head and Neck Surgery. 1995 Oct;113(4):435-9. https://doi.org/10.1016/S0194-59989570081-1</ref><ref name="Huang 2014">Huang Y, Liu Z, Wang J, Sun X, Yang L, Wang D. Surgical management of juvenile nasopharyngeal angiofibroma: analysis of 162 cases from 1995 to 2012. The Laryngoscope. 2014 Aug;124(8):1942-6. https://doi.org/10.1002/lary.24522</ref> It classically presents in adolescent males, with a typical age of presentation between 13 and 22 years old.<ref name ="Newman 2023">Newman M, Nguyen TB, McHugh T, Reddy K, Sommer DD. Early-onset juvenile nasopharyngeal angiofibroma (JNA): a systematic review. Journal of Otolaryngology-Head & Neck Surgery. 2023 Dec;52(1):s40463-023. https://doi.org/10.1186/s40463-023-00687-w</ref>

=== Genetics ===
There is no identified definitive genetic locus that predisposes patients to JNA's. However, there have been a number of studies looking at the various genetic components that could influence their formation.<ref name="Doody 2019">Doody J, Adil EA, Trenor III CC, Cunningham MJ. The genetic and molecular determinants of juvenile nasopharyngeal angiofibroma: a systematic review. Annals of Otology, Rhinology & Laryngology. 2019 Nov;128(11):1061-72. https://doi.org/10.1177/0003489419850194</ref> Due to fact that JNA's classically form in adolescent males, there have been several studies focused on the possible hormonal influence on their formation and growth. JNA's tend to have an abundance of progesterone and estradiol, but a relatively lower concentration of testosterone and dihydrotestosterone.<ref name="Kumagami 05 1993">Kumagami H. Estradiol, dihydrotestosterone, and testosterone in juvenile nasopharyngeal angiofibroma tissue. American Journal of Rhinology. 1993 May;7(3):101-4. https://doi.org/10.2500/105065893781976393</ref><ref name="Kumagami 01 1993">Kumagami H. Sex hormones in juvenile nasopharyngeal angiofibroma tissue. Auris Nasus Larynx. 1993 Jan 1;20(2):131-5. https://doi.org/10.1016/S0385-8146(12)80240-9</ref> Supplemental testosterone was trialed in JNA patients, but both tumor growth<ref name="Johnsen 1966">Johnsen S, Kloster JH, Schiff M. The action of hormones on juvenile nasopharyngeal angiofibroma. Acta Oto-Laryngologica. 1966 Jan 1;61(1-6):153-60. https://doi.org/10.3109/00016486609127052</ref> and tumor recurrence<ref name="Riggs 2010">Riggs S, Orlandi RR. Juvenile nasopharyngeal angiofibroma recurrence associated with exogenous testosterone therapy. Head & Neck: Journal for the Sciences and Specialties of the Head and Neck. 2010 Jun;32(6):812-5. https://doi.org/10.1002/hed.21152</ref> have been documented. Papers investigating potential targeted therapy markers have looked at steroid hormones, chromosomal abnormalities, growth factors, and other intracellular molecular targets.<ref name="Doody 2019"></ref> There have been studies investigating a possible link between Familial Adenomatous Polyposis (FAP) and JNA's via the adenomatous polyposis coli (APC)/β-catenin pathway, but no specific genetic link has been identified as of yet.<ref name="Guertl 2000">Guertl B, Beham A, Zechner R, Stammberger H, Hoefler G. Nasopharyngeal angiofibroma: an AM-Gene-Associated tumor?. Human pathology. 2000 Nov 1;31(11):1411-3.</ref>

=== Histology ===
Grossly on histologic section there are large fibrous/collagenous sections with fibroblasts, as well as vascular spaces of various sizes throughout the tumor.<ref name="Xu 2020">Xu B. Nasopharyngeal angiofibroma. PathologyOutlines.com website. https://www.pathologyoutlines.com/topic/nasalangiofibroma.html. Accessed November 17th, 2024.</ref> The stromal cells have an abundance of beta-catenin relative to the vascular endothelial cells.

<gallery>
JNA 30x.png|H&E stained section of green inked vascular channel (30x)
Beta Catenin JNA.png|Abnormal nuclear expression in stromal cells (blue arrow), compared with membranous / cytoplasmic staining in the endothelial cells (red arrow)
</gallery>

== Diagnosis ==
=== Patient History ===
Patients may have a history of any of the following symptoms:
* Unilateral nasal obstruction
* Recurrent epistaxis
* High-volume epistaxis
* Eustachian tube dysfunction
* Headache
* Facial swelling
* Anosmia / Hyposmia
* Cranial neuropathy
* Vision changes

=== Physical Examination ===
Many patients will not have obvious findings on anterior rhinoscopy. A large vascular-appearing mass is commonly fond on nasal endoscopy on the posterior lateral nasal sidewall near the region of the sphenopalatine artery. Note that '''''these hypervascular masses should not be biopsied''''', as this may result in significant bleeding that is hard to control in the clinic setting.

=== Laboratory Tests ===
Laboratory tests are not commonly useful in the diagnosis of JNA's, but should be performed preoperatively in the case of a planned surgical resection.
* Hemoglobin / Hematocrit
* Platelet level
* Platelet function (if family history of platelet dysfunction)
* PT / INR
* Blood Type and Screen (all patients)
* Blood Type and Cross (patients with advanced stage tumors where a significant blood loss is expected)

=== Imaging ===
[[File:Holman-Miller Sign.png|right|thumb|300 px|Axial CT scan of a JNA showing a right-sided Holman Miller sign]]
CT scan, angiography, and MRI are all useful in the workup of JNA's and have differing benefits.<ref name="Gaillard 2008">Gaillard F, Walizai T, Bell D, et al. Juvenile nasopharyngeal angiofibroma. Reference article, Radiopaedia.org (Accessed on 17 Nov 2024) https://doi.org/10.53347/rID-1541</ref> CT is typically the first imaging modality ordered in these patients, and is helpful for delineating the extent of bony erosion. Angiography demonstrates the extent of the mass well, and is frequently used in pre-operative embolization 24-48 hours before surgical resection. A majority of JNA's are supplied by the external carotid system, either by the maxillary artery (SPA) or the ascending pharyngeal artery.<ref name="Gaillard 2008"></ref> A lesser fraction of tumors are supplied by the internal carotid system (ophthalmic or sphenoidal branches), and this is typically in larger masses with intracranial invasion. MRI can be used to assess for intraorbital or intracranial extension. JNA's are T1 intermediate, T2 heterogenous with flow voids, and enhance with Gadolinium. The following are general characteristics often found in imaging of JNA's:
* Highly vascular nasopharyngeal mass
* Holman-Miller Sign<ref name="Niknejad 2013">Niknejad M, Tatco V, Bell D, et al. Holman-Miller sign (maxillary sinus). Reference article, Radiopaedia.org (Accessed on 17 Nov 2024) https://doi.org/10.53347/rID-21804</ref>: Anterior displacement of the posterior maxillary wall due to mass effect from the tumor
* Widening of the PMF or PPF
* Erosion of the pterygoid plate, especially the medial plate

There are several classification systems that have been proposed for JNAs based on imaging findings.

{| class="wikitable", style="margin-left: auto; margin-right: auto; border: none; text-align: center"
|+ Classification Systems for Juvenile Nasopharyngeal Angiofibromas
|-
! Classification System !! style=width:15em | Stage I !! style=width:15em | Stage II !! style=width:15em | Stage III !! style=width:15em | Stage IV !! style=width:15em | Stage V
|-
| Andrews (1989)<ref name="Andrews 1989">Andrews JC, Fisch U, Aeppli U, Valavanis A, Makek MS. The surgical management of extensive nasopharyngeal angiofibromas with the infratemporal fossa approach. The Laryngoscope. 1989 Apr;99(4):429-37. https://doi.org/10.1288/00005537-198904000-00013</ref> || Confined to NP || Invading one of the following with evidence of bony erosion: PPF, MS, ES, or SS || Invading ITF or orbit '''IIIa''': No intracranial extension '''IIIb''': Extradural (parasellar) extension || Intradural extension '''IVa''': No infiltration of CS, PF, or OC '''IVb''': Infiltration of CS, PF, or OC || --
|-
| Chandler (1984)<ref name="Chandler 1984">Chandler JR, Moskowitz L, Goulding R, Quencer RM. Nasopharyngeal angiofibromas: staging and management. Annals of Otology, Rhinology & Laryngology. 1984 Jul;93(4):322-9. https://doi.org/10.1177/000348948409300408</ref> || Confined to NP || Extension into the nasal cavity, SS, or both || Extension into any of the following: antrum, ES, PMF, ITF, orbit, or cheek || Intracranial extension || --
|-
| Onerci (2006)<ref name="Onerci 2006">Onerci M, Oğretmenoğlu O, Yücel T. Juvenile nasopharyngeal angiofibroma: a revised staging system. Rhinology. 2006 Mar 1;44(1):39-45. PMID: 16550949</ref>|| Nose, NP, ES, and SS or minimal extension into PMF || MS involvement, full occupation of PMF, extension to anterior cranial fossa, limited extension into ITF || Deep extension into cancellous bone at pterygoid base or body and GW of sphenoid, significant lateral extension into ITF or pterygoid plates, orbital involvement, CS obliteration || Intracranial extension between pituitary gland and ICA, tumor localization lateral to ICA, middle fossa extension and extensive intracranial extension || --
|-
| Radkowski (1996)<ref name="Radkowski 1996">Radkowski D, McGill T, Healy GB, Ohlms L, Jones DT. Angiofibroma: changes in staging and treatment. Archives of Otolaryngology–Head & Neck Surgery. 1996 Feb 1;122(2):122-9. https://doi.org/10.1001/archotol.1996.01890140012004</ref>|| '''IA''': Confined to nose or NP '''IB''': Extends into one or more sinuses || '''IIa''': Minimal extension into medial PMF '''IIb''': Full occupation of PMF with local mass effect '''IIc''': Extension into ITF, cheek, or posterior to pterygoid plates || Erosion of skull base '''IIIa''': Minimal skull base involvement '''IIIb''': Extensive intracranial extension, with or without invasion into CS || -- || --
|-
| Sessions (1981)<ref name="Sessions 1981">Sessions RB, Bryan RN, Naclerio RM, Alford BR. Radiographic staging of juvenile angiofibroma. Head & neck surgery. 1981 Mar;3(4):279-83. https://doi.org/10.1002/hed.2890030404</ref> || '''IA''': Confined to nose or NP '''IB''': Extends into one or more sinuses || '''IIa''': Minimal extension into PMF '''IIb''': Full occupation of PMF with or without orbital erosion '''IIc''': ITF with or without cheek extension || Intracranial extension || -- || --
|-
| UPMC (2010)<ref name="Snyderman 2010">Snyderman CH, Pant H, Carrau RL, Gardner P. A new endoscopic staging system for angiofibromas. Archives of Otolaryngology–Head & Neck Surgery. 2010 Jun 21;136(6):588-94. https://doi.org/10.1001/archoto.2010.83</ref> || Nasal cavity, medial PPF || Paranasal sinuses, lateral PPF; no residual vascularity || Skull base erosion, orbit, ITF involvement; no residual vascularity || Skull base erosion, orbit, ITF involvement; residual vascularity || Intracranial extension with residual vascularity '''M''': Medial extension '''L''': Lateral extension
|-
|+ style="caption-side:bottom; font-weight: normal;"|''Abbreviations: Cavernous sinus (CS), Ethmoid sinus (ES), Infratemporal fossa (ITF), Internal carotid artery (ICA), Maxillary sinus (MS), Nasopharynx (NP), Optic chiasm (OC), Pituitary fossa (PF), Pterygomaxillary fissure (PMF), Pterygopalatine fossa (PPF), Sphenoid sinus (SS)''
|}

=== Differential Diagnosis ===
In the case of a nasopharyngeal mass, the following should also be considered:
* Clival chondroma
* Encephalocele
* Esthesioneuroblastoma
* Inverted papilloma
* Nasal polyp
* Nasopharyngeal carcinoma
* Nasopharyngeal teratoma
* Rhabdomyosarcoma
* Vascular malformation

== Management ==
=== Medical Management ===
There is no approved medical treatment for management of juvenile nasopharyngeal angiofibromas. The treatment is primarily surgical. Medical management focuses on optimizing patients before surgery, such as addressing any coagulopathies or addressing underlying anemia.

=== Surgical Management ===
Historically, surgical management of JNAs involved aggressive open approaches, including transpalatal, lateral rhinotomy, midface degloving, or infratemporal fossa craniotomy approaches.<ref name="Renkonen 2011">Renkonen S, Hagström J, Vuola J, Niemelä M, Porras M, Kivivuori SM, Leivo I, Mäkitie AA. The changing surgical management of juvenile nasopharyngeal angiofibroma. European archives of oto-rhino-laryngology. 2011 Apr;268:599-607. https://doi.org/10.1007/s00405-010-1383-z</ref> In recent years, there has been a transition towards transnasal endoscopic approaches. This has resulted in better visualization during surgery and a subsequent improvement in intraoperative blood loss, need for blood transfusions,<ref name="Oliveira 2012">Oliveira JA, Tavares MG, Aguiar CV, Azevedo JF, Sousa JR, Almeida PC, Gomes EF. Comparison between endoscopic and open surgery in 37 patients with nasopharyngeal angiofibroma. Brazilian Journal of otorhinolaryngology. 2012;78:75-80. https://doi.org/10.1590/S1808-86942012000100012</ref> and recurrence rates.<ref name="Bosraty 2011">Bosraty H, Atef A, Aziz M. Endoscopic vs. open surgery for treating large, locally advanced juvenile angiofibromas: A comparison of local control and morbidity outcomes. ENT: Ear, Nose & Throat Journal. 2011 Nov 1;90(11). PMID: 22109921</ref> Prior to surgery, preoperative embolization should be considered to limit blood loss intraoperatively.

== Outcomes ==
=== Complications ===
=== Prognosis ===
Recurrence rates can be very high and increase with increased stage at the time of surgery. Recurrence has been reported from 22%<ref name="Bleier 2009">Bleier BS, Kennedy DW, Palmer JN, Chiu AG, Bloom JD, O'Malley Jr BW. Current management of juvenile nasopharyngeal angiofibroma: a tertiary center experience 1999–2007. American journal of rhinology & allergy. 2009 May;23(3):328-30. https://doi.org/10.2500/ajra.2009.23.3322</ref> to as high as 50% in advanced disease.<ref name="Onerci 2003">Önerci TM, Yücel ÖT, Öğretmenoğlu O. Endoscopic surgery in treatment of juvenile nasopharyngeal angiofibroma. International journal of pediatric otorhinolaryngology. 2003 Nov 1;67(11):1219-25. https://doi.org/10.1016/j.ijporl.2003.07.013</ref>

== References ==
<references />

Juvenile Nasopharyngeal Angiofibroma

2024-11-17T21:30:06Z

Jack.Dewey: /* Surgical Management */

{{infobox Disease
|Title =
|Aliases =
|Image = [[File:Nasopharyngeal angiofibroma - 2 - high mag.jpg]]
|Caption = Histologic section of a JNA
|ICD-9 = 210.7
|ICD-10 = D10.6
|MeSH =
|Gene =
|Locus =
|OMIM =
|EyeWiki =
|Radiopaedia = [https://radiopaedia.org/articles/juvenile-nasopharyngeal-angiofibroma?lang=us Juvenile Nasopharyngeal Angiofibroma]
|Pathology = [https://www.pathologyoutlines.com/topic/nasalangiofibroma.html Nasal Angiofibroma]
}}

== Overview ==
'''Juvenile nasopharyngeal angiofibroma (JNA)''' is a benign vascular neoplasm of the nasal cavity and nasopharynx that classically presents in adolescent boys.

== Pathophysiology ==
=== Relevant Anatomy ===
JNA's typically originate in the region of the sphenopalatine artery (SPA) in the posterior nasal cavity/nasopharynx. From there, they can extend into the paranasal sinuses, pterygopalatine fossa (PPF), infratemporal fossa (ITF), orbit, or cranial vault.

<gallery>
Pterygopalatine fossa.jpg|Cadaveric model of the PPF
Schematic diagram showing the bony anatomy and main neural connections of the PPF.png|Neural contents of the PPF
Gray511.png|Maxillary artery branches, with SPA at the distal end
Gerrish's Text-book of Anatomy (1902) - Fig. 243.png|Location of the sphenopalatine foramen
Basilar process and palatine process.jpg|Location of the ITF
</gallery>

=== Disease Etiology ===
JNA's are fibrovascular tumors that arise from the posterior lateral nasal cavity near the choana. There is substantial debate about the exact circumstances and location that lead to the formation of a JNA, but they are generally accepted to form near the region of the sphenopalatine foramen. Proposed theories regarding their etiology include androgen receptor-related growth,<ref name="Liu 2015">Liu Z, Wang J, Wang H, Wang D, Hu L, Liu Q, Sun X. Hormonal receptors and vascular endothelial growth factor in juvenile nasopharyngeal angiofibroma: immunohistochemical and tissue microarray analysis. Acta oto-laryngologica. 2015 Jan 2;135(1):51-7. https://doi.org/10.3109/00016489.2014.952774</ref> incomplete regression of a branchial artery during embryogenesis,<ref name="Schick 2002">Schick B, Plinkert PK, Prescher A. Aetiology of angiofibromas: reflection on their specific vascular component. Laryngo-rhino-otologie. 2002 Apr 1;81(4):280-4. https://doi.org/10.1055/s-2002-25322</ref> oncogenic mutations in C-MYC and C-KIT,<ref name="Pandey 2017">Pandey P, Mishra A, Tripathi AM, Verma V, Trivedi R, Singh HP, Kumar S, Patel B, Singh V, Pandey S, Pandey A. Current molecular profile of juvenile nasopharyngeal angiofibroma: first comprehensive study from India. The Laryngoscope. 2017 Mar;127(3):E100-6. https://doi.org/10.1002/lary.26250</ref> and a wide variety of other genetic aberrations.

===Epidemiology===
Juvenile nasopharyngeal angiofibroma has been reported to be the most common benign neoplasm of the nasopharynx in young males. It accounts for 0.05% to 0.5% of all head and neck tumors, with a reported incidence of 1 in 5,000 to 1 in 60,000 in the US annually.<ref name="Boghani 2013">Boghani Z, Husain Q, Kanumuri VV, Khan MN, Sangvhi S, Liu JK, Eloy JA. Juvenile nasopharyngeal angiofibroma: a systematic review and comparison of endoscopic, endoscopic‐assisted, and open resection in 1047 cases. The Laryngoscope. 2013 Apr;123(4):859-69. https://doi.org/10.1002/lary.23843</ref><ref name="Ferouz 1995">Ferouz AS, Mohr RM, Paul P. Juvenile nasopharyngeal angiofibroma and familial adenomatous polyposis: an association?. Otolaryngology—Head and Neck Surgery. 1995 Oct;113(4):435-9. https://doi.org/10.1016/S0194-59989570081-1</ref><ref name="Huang 2014">Huang Y, Liu Z, Wang J, Sun X, Yang L, Wang D. Surgical management of juvenile nasopharyngeal angiofibroma: analysis of 162 cases from 1995 to 2012. The Laryngoscope. 2014 Aug;124(8):1942-6. https://doi.org/10.1002/lary.24522</ref> It classically presents in adolescent males, with a typical age of presentation between 13 and 22 years old.<ref name ="Newman 2023">Newman M, Nguyen TB, McHugh T, Reddy K, Sommer DD. Early-onset juvenile nasopharyngeal angiofibroma (JNA): a systematic review. Journal of Otolaryngology-Head & Neck Surgery. 2023 Dec;52(1):s40463-023. https://doi.org/10.1186/s40463-023-00687-w</ref>

=== Genetics ===
There is no identified definitive genetic locus that predisposes patients to JNA's. However, there have been a number of studies looking at the various genetic components that could influence their formation.<ref name="Doody 2019">Doody J, Adil EA, Trenor III CC, Cunningham MJ. The genetic and molecular determinants of juvenile nasopharyngeal angiofibroma: a systematic review. Annals of Otology, Rhinology & Laryngology. 2019 Nov;128(11):1061-72. https://doi.org/10.1177/0003489419850194</ref> Due to fact that JNA's classically form in adolescent males, there have been several studies focused on the possible hormonal influence on their formation and growth. JNA's tend to have an abundance of progesterone and estradiol, but a relatively lower concentration of testosterone and dihydrotestosterone.<ref name="Kumagami 05 1993">Kumagami H. Estradiol, dihydrotestosterone, and testosterone in juvenile nasopharyngeal angiofibroma tissue. American Journal of Rhinology. 1993 May;7(3):101-4. https://doi.org/10.2500/105065893781976393</ref><ref name="Kumagami 01 1993">Kumagami H. Sex hormones in juvenile nasopharyngeal angiofibroma tissue. Auris Nasus Larynx. 1993 Jan 1;20(2):131-5. https://doi.org/10.1016/S0385-8146(12)80240-9</ref> Supplemental testosterone was trialed in JNA patients, but both tumor growth<ref name="Johnsen 1966">Johnsen S, Kloster JH, Schiff M. The action of hormones on juvenile nasopharyngeal angiofibroma. Acta Oto-Laryngologica. 1966 Jan 1;61(1-6):153-60. https://doi.org/10.3109/00016486609127052</ref> and tumor recurrence<ref name="Riggs 2010">Riggs S, Orlandi RR. Juvenile nasopharyngeal angiofibroma recurrence associated with exogenous testosterone therapy. Head & Neck: Journal for the Sciences and Specialties of the Head and Neck. 2010 Jun;32(6):812-5. https://doi.org/10.1002/hed.21152</ref> have been documented. Papers investigating potential targeted therapy markers have looked at steroid hormones, chromosomal abnormalities, growth factors, and other intracellular molecular targets.<ref name="Doody 2019"></ref> There have been studies investigating a possible link between Familial Adenomatous Polyposis (FAP) and JNA's via the adenomatous polyposis coli (APC)/β-catenin pathway, but no specific genetic link has been identified as of yet.<ref name="Guertl 2000">Guertl B, Beham A, Zechner R, Stammberger H, Hoefler G. Nasopharyngeal angiofibroma: an AM-Gene-Associated tumor?. Human pathology. 2000 Nov 1;31(11):1411-3.</ref>

=== Histology ===
Grossly on histologic section there are large fibrous/collagenous sections with fibroblasts, as well as vascular spaces of various sizes throughout the tumor.<ref name="Xu 2020">Xu B. Nasopharyngeal angiofibroma. PathologyOutlines.com website. https://www.pathologyoutlines.com/topic/nasalangiofibroma.html. Accessed November 17th, 2024.</ref> The stromal cells have an abundance of beta-catenin relative to the vascular endothelial cells.

<gallery>
JNA 30x.png|H&E stained section of green inked vascular channel (30x)
Beta Catenin JNA.png|Abnormal nuclear expression in stromal cells (blue arrow), compared with membranous / cytoplasmic staining in the endothelial cells (red arrow)
</gallery>

== Diagnosis ==
=== Patient History ===
Patients may have a history of any of the following symptoms:
* Unilateral nasal obstruction
* Recurrent epistaxis
* High-volume epistaxis
* Eustachian tube dysfunction
* Headache
* Facial swelling
* Anosmia / Hyposmia
* Cranial neuropathy
* Vision changes

=== Physical Examination ===
Many patients will not have obvious findings on anterior rhinoscopy. A large vascular-appearing mass is commonly fond on nasal endoscopy on the posterior lateral nasal sidewall near the region of the sphenopalatine artery. Note that '''''these hypervascular masses should not be biopsied''''', as this may result in significant bleeding that is hard to control in the clinic setting.

=== Laboratory Tests ===
Laboratory tests are not commonly useful in the diagnosis of JNA's, but should be performed preoperatively in the case of a planned surgical resection.
* Hemoglobin / Hematocrit
* Platelet level
* Platelet function (if family history of platelet dysfunction)
* PT / INR
* Blood Type and Screen (all patients)
* Blood Type and Cross (patients with advanced stage tumors where a significant blood loss is expected)

=== Imaging ===
[[File:Holman-Miller Sign.png|right|thumb|300 px|Axial CT scan of a JNA showing a right-sided Holman Miller sign]]
CT scan, angiography, and MRI are all useful in the workup of JNA's and have differing benefits.<ref name="Gaillard 2008">Gaillard F, Walizai T, Bell D, et al. Juvenile nasopharyngeal angiofibroma. Reference article, Radiopaedia.org (Accessed on 17 Nov 2024) https://doi.org/10.53347/rID-1541</ref> CT is typically the first imaging modality ordered in these patients, and is helpful for delineating the extent of bony erosion. Angiography demonstrates the extent of the mass well, and is frequently used in pre-operative embolization 24-48 hours before surgical resection. A majority of JNA's are supplied by the external carotid system, either by the maxillary artery (SPA) or the ascending pharyngeal artery.<ref name="Gaillard 2008"></ref> A lesser fraction of tumors are supplied by the internal carotid system (ophthalmic or sphenoidal branches), and this is typically in larger masses with intracranial invasion. MRI can be used to assess for intraorbital or intracranial extension. JNA's are T1 intermediate, T2 heterogenous with flow voids, and enhance with Gadolinium. The following are general characteristics often found in imaging of JNA's:
* Highly vascular nasopharyngeal mass
* Holman-Miller Sign<ref name="Niknejad 2013">Niknejad M, Tatco V, Bell D, et al. Holman-Miller sign (maxillary sinus). Reference article, Radiopaedia.org (Accessed on 17 Nov 2024) https://doi.org/10.53347/rID-21804</ref>: Anterior displacement of the posterior maxillary wall due to mass effect from the tumor
* Widening of the PMF or PPF
* Erosion of the pterygoid plate, especially the medial plate

There are several classification systems that have been proposed for JNAs based on imaging findings.

{| class="wikitable", style="margin-left: auto; margin-right: auto; border: none; text-align: center"
|+ Classification Systems for Juvenile Nasopharyngeal Angiofibromas
|-
! Classification System !! style=width:15em | Stage I !! style=width:15em | Stage II !! style=width:15em | Stage III !! style=width:15em | Stage IV !! style=width:15em | Stage V
|-
| Andrews (1989)<ref name="Andrews 1989">Andrews JC, Fisch U, Aeppli U, Valavanis A, Makek MS. The surgical management of extensive nasopharyngeal angiofibromas with the infratemporal fossa approach. The Laryngoscope. 1989 Apr;99(4):429-37. https://doi.org/10.1288/00005537-198904000-00013</ref> || Confined to NP || Invading one of the following with evidence of bony erosion: PPF, MS, ES, or SS || Invading ITF or orbit '''IIIa''': No intracranial extension '''IIIb''': Extradural (parasellar) extension || Intradural extension '''IVa''': No infiltration of CS, PF, or OC '''IVb''': Infiltration of CS, PF, or OC || --
|-
| Chandler (1984)<ref name="Chandler 1984">Chandler JR, Moskowitz L, Goulding R, Quencer RM. Nasopharyngeal angiofibromas: staging and management. Annals of Otology, Rhinology & Laryngology. 1984 Jul;93(4):322-9. https://doi.org/10.1177/000348948409300408</ref> || Confined to NP || Extension into the nasal cavity, SS, or both || Extension into any of the following: antrum, ES, PMF, ITF, orbit, or cheek || Intracranial extension || --
|-
| Onerci (2006)<ref name="Onerci 2006">Onerci M, Oğretmenoğlu O, Yücel T. Juvenile nasopharyngeal angiofibroma: a revised staging system. Rhinology. 2006 Mar 1;44(1):39-45. PMID: 16550949</ref>|| Nose, NP, ES, and SS or minimal extension into PMF || MS involvement, full occupation of PMF, extension to anterior cranial fossa, limited extension into ITF || Deep extension into cancellous bone at pterygoid base or body and GW of sphenoid, significant lateral extension into ITF or pterygoid plates, orbital involvement, CS obliteration || Intracranial extension between pituitary gland and ICA, tumor localization lateral to ICA, middle fossa extension and extensive intracranial extension || --
|-
| Radkowski (1996)<ref name="Radkowski 1996">Radkowski D, McGill T, Healy GB, Ohlms L, Jones DT. Angiofibroma: changes in staging and treatment. Archives of Otolaryngology–Head & Neck Surgery. 1996 Feb 1;122(2):122-9. https://doi.org/10.1001/archotol.1996.01890140012004</ref>|| '''IA''': Confined to nose or NP '''IB''': Extends into one or more sinuses || '''IIa''': Minimal extension into medial PMF '''IIb''': Full occupation of PMF with local mass effect '''IIc''': Extension into ITF, cheek, or posterior to pterygoid plates || Erosion of skull base '''IIIa''': Minimal skull base involvement '''IIIb''': Extensive intracranial extension, with or without invasion into CS || -- || --
|-
| Sessions (1981)<ref name="Sessions 1981">Sessions RB, Bryan RN, Naclerio RM, Alford BR. Radiographic staging of juvenile angiofibroma. Head & neck surgery. 1981 Mar;3(4):279-83. https://doi.org/10.1002/hed.2890030404</ref> || '''IA''': Confined to nose or NP '''IB''': Extends into one or more sinuses || '''IIa''': Minimal extension into PMF '''IIb''': Full occupation of PMF with or without orbital erosion '''IIc''': ITF with or without cheek extension || Intracranial extension || -- || --
|-
| UPMC (2010)<ref name="Snyderman 2010">Snyderman CH, Pant H, Carrau RL, Gardner P. A new endoscopic staging system for angiofibromas. Archives of Otolaryngology–Head & Neck Surgery. 2010 Jun 21;136(6):588-94. https://doi.org/10.1001/archoto.2010.83</ref> || Nasal cavity, medial PPF || Paranasal sinuses, lateral PPF; no residual vascularity || Skull base erosion, orbit, ITF involvement; no residual vascularity || Skull base erosion, orbit, ITF involvement; residual vascularity || Intracranial extension with residual vascularity '''M''': Medial extension '''L''': Lateral extension
|-
|+ style="caption-side:bottom; font-weight: normal;"|''Abbreviations: Cavernous sinus (CS), Ethmoid sinus (ES), Infratemporal fossa (ITF), Internal carotid artery (ICA), Maxillary sinus (MS), Nasopharynx (NP), Optic chiasm (OC), Pituitary fossa (PF), Pterygomaxillary fissure (PMF), Pterygopalatine fossa (PPF), Sphenoid sinus (SS)''
|}

=== Differential Diagnosis ===
In the case of a nasopharyngeal mass, the following should also be considered:
* Clival chondroma
* Encephalocele
* Esthesioneuroblastoma
* Inverted papilloma
* Nasal polyp
* Nasopharyngeal carcinoma
* Nasopharyngeal teratoma
* Rhabdomyosarcoma
* Vascular malformation

== Management ==
=== Medical Management ===
There is no approved medical treatment for management of juvenile nasopharyngeal angiofibromas. The treatment is primarily surgical. Medical management focuses on optimizing patients before surgery, such as addressing any coagulopathies or addressing underlying anemia.

=== Surgical Management ===
Historically, surgical management of JNAs involved aggressive open approaches, including transpalatal, lateral rhinotomy, midface degloving, or infratemporal fossa craniotomy approaches.<ref name="Renkonen 2011">Renkonen S, Hagström J, Vuola J, Niemelä M, Porras M, Kivivuori SM, Leivo I, Mäkitie AA. The changing surgical management of juvenile nasopharyngeal angiofibroma. European archives of oto-rhino-laryngology. 2011 Apr;268:599-607. https://doi.org/10.1007/s00405-010-1383-z</ref> In recent years, there has been a transition towards transnasal endoscopic approaches. This has resulted in better visualization during surgery and a subsequent improvement in intraoperative blood loss, need for blood transfusions,<ref name="Oliveira 2012">Oliveira JA, Tavares MG, Aguiar CV, Azevedo JF, Sousa JR, Almeida PC, Gomes EF. Comparison between endoscopic and open surgery in 37 patients with nasopharyngeal angiofibroma. Brazilian Journal of otorhinolaryngology. 2012;78:75-80. https://doi.org/10.1590/S1808-86942012000100012</ref> and recurrence rates.<ref name="Bosraty 2011">Bosraty H, Atef A, Aziz M. Endoscopic vs. open surgery for treating large, locally advanced juvenile angiofibromas: A comparison of local control and morbidity outcomes. ENT: Ear, Nose & Throat Journal. 2011 Nov 1;90(11). PMID: 22109921</ref> Prior to surgery, preoperative embolization should be considered to limit blood loss intraoperatively.

== Outcomes ==
=== Complications ===
=== Prognosis ===

== References ==
<references />

Juvenile Nasopharyngeal Angiofibroma

2024-11-17T21:29:29Z

Jack.Dewey: /* Surgical Management */

{{infobox Disease
|Title =
|Aliases =
|Image = [[File:Nasopharyngeal angiofibroma - 2 - high mag.jpg]]
|Caption = Histologic section of a JNA
|ICD-9 = 210.7
|ICD-10 = D10.6
|MeSH =
|Gene =
|Locus =
|OMIM =
|EyeWiki =
|Radiopaedia = [https://radiopaedia.org/articles/juvenile-nasopharyngeal-angiofibroma?lang=us Juvenile Nasopharyngeal Angiofibroma]
|Pathology = [https://www.pathologyoutlines.com/topic/nasalangiofibroma.html Nasal Angiofibroma]
}}

== Overview ==
'''Juvenile nasopharyngeal angiofibroma (JNA)''' is a benign vascular neoplasm of the nasal cavity and nasopharynx that classically presents in adolescent boys.

== Pathophysiology ==
=== Relevant Anatomy ===
JNA's typically originate in the region of the sphenopalatine artery (SPA) in the posterior nasal cavity/nasopharynx. From there, they can extend into the paranasal sinuses, pterygopalatine fossa (PPF), infratemporal fossa (ITF), orbit, or cranial vault.

<gallery>
Pterygopalatine fossa.jpg|Cadaveric model of the PPF
Schematic diagram showing the bony anatomy and main neural connections of the PPF.png|Neural contents of the PPF
Gray511.png|Maxillary artery branches, with SPA at the distal end
Gerrish's Text-book of Anatomy (1902) - Fig. 243.png|Location of the sphenopalatine foramen
Basilar process and palatine process.jpg|Location of the ITF
</gallery>

=== Disease Etiology ===
JNA's are fibrovascular tumors that arise from the posterior lateral nasal cavity near the choana. There is substantial debate about the exact circumstances and location that lead to the formation of a JNA, but they are generally accepted to form near the region of the sphenopalatine foramen. Proposed theories regarding their etiology include androgen receptor-related growth,<ref name="Liu 2015">Liu Z, Wang J, Wang H, Wang D, Hu L, Liu Q, Sun X. Hormonal receptors and vascular endothelial growth factor in juvenile nasopharyngeal angiofibroma: immunohistochemical and tissue microarray analysis. Acta oto-laryngologica. 2015 Jan 2;135(1):51-7. https://doi.org/10.3109/00016489.2014.952774</ref> incomplete regression of a branchial artery during embryogenesis,<ref name="Schick 2002">Schick B, Plinkert PK, Prescher A. Aetiology of angiofibromas: reflection on their specific vascular component. Laryngo-rhino-otologie. 2002 Apr 1;81(4):280-4. https://doi.org/10.1055/s-2002-25322</ref> oncogenic mutations in C-MYC and C-KIT,<ref name="Pandey 2017">Pandey P, Mishra A, Tripathi AM, Verma V, Trivedi R, Singh HP, Kumar S, Patel B, Singh V, Pandey S, Pandey A. Current molecular profile of juvenile nasopharyngeal angiofibroma: first comprehensive study from India. The Laryngoscope. 2017 Mar;127(3):E100-6. https://doi.org/10.1002/lary.26250</ref> and a wide variety of other genetic aberrations.

===Epidemiology===
Juvenile nasopharyngeal angiofibroma has been reported to be the most common benign neoplasm of the nasopharynx in young males. It accounts for 0.05% to 0.5% of all head and neck tumors, with a reported incidence of 1 in 5,000 to 1 in 60,000 in the US annually.<ref name="Boghani 2013">Boghani Z, Husain Q, Kanumuri VV, Khan MN, Sangvhi S, Liu JK, Eloy JA. Juvenile nasopharyngeal angiofibroma: a systematic review and comparison of endoscopic, endoscopic‐assisted, and open resection in 1047 cases. The Laryngoscope. 2013 Apr;123(4):859-69. https://doi.org/10.1002/lary.23843</ref><ref name="Ferouz 1995">Ferouz AS, Mohr RM, Paul P. Juvenile nasopharyngeal angiofibroma and familial adenomatous polyposis: an association?. Otolaryngology—Head and Neck Surgery. 1995 Oct;113(4):435-9. https://doi.org/10.1016/S0194-59989570081-1</ref><ref name="Huang 2014">Huang Y, Liu Z, Wang J, Sun X, Yang L, Wang D. Surgical management of juvenile nasopharyngeal angiofibroma: analysis of 162 cases from 1995 to 2012. The Laryngoscope. 2014 Aug;124(8):1942-6. https://doi.org/10.1002/lary.24522</ref> It classically presents in adolescent males, with a typical age of presentation between 13 and 22 years old.<ref name ="Newman 2023">Newman M, Nguyen TB, McHugh T, Reddy K, Sommer DD. Early-onset juvenile nasopharyngeal angiofibroma (JNA): a systematic review. Journal of Otolaryngology-Head & Neck Surgery. 2023 Dec;52(1):s40463-023. https://doi.org/10.1186/s40463-023-00687-w</ref>

=== Genetics ===
There is no identified definitive genetic locus that predisposes patients to JNA's. However, there have been a number of studies looking at the various genetic components that could influence their formation.<ref name="Doody 2019">Doody J, Adil EA, Trenor III CC, Cunningham MJ. The genetic and molecular determinants of juvenile nasopharyngeal angiofibroma: a systematic review. Annals of Otology, Rhinology & Laryngology. 2019 Nov;128(11):1061-72. https://doi.org/10.1177/0003489419850194</ref> Due to fact that JNA's classically form in adolescent males, there have been several studies focused on the possible hormonal influence on their formation and growth. JNA's tend to have an abundance of progesterone and estradiol, but a relatively lower concentration of testosterone and dihydrotestosterone.<ref name="Kumagami 05 1993">Kumagami H. Estradiol, dihydrotestosterone, and testosterone in juvenile nasopharyngeal angiofibroma tissue. American Journal of Rhinology. 1993 May;7(3):101-4. https://doi.org/10.2500/105065893781976393</ref><ref name="Kumagami 01 1993">Kumagami H. Sex hormones in juvenile nasopharyngeal angiofibroma tissue. Auris Nasus Larynx. 1993 Jan 1;20(2):131-5. https://doi.org/10.1016/S0385-8146(12)80240-9</ref> Supplemental testosterone was trialed in JNA patients, but both tumor growth<ref name="Johnsen 1966">Johnsen S, Kloster JH, Schiff M. The action of hormones on juvenile nasopharyngeal angiofibroma. Acta Oto-Laryngologica. 1966 Jan 1;61(1-6):153-60. https://doi.org/10.3109/00016486609127052</ref> and tumor recurrence<ref name="Riggs 2010">Riggs S, Orlandi RR. Juvenile nasopharyngeal angiofibroma recurrence associated with exogenous testosterone therapy. Head & Neck: Journal for the Sciences and Specialties of the Head and Neck. 2010 Jun;32(6):812-5. https://doi.org/10.1002/hed.21152</ref> have been documented. Papers investigating potential targeted therapy markers have looked at steroid hormones, chromosomal abnormalities, growth factors, and other intracellular molecular targets.<ref name="Doody 2019"></ref> There have been studies investigating a possible link between Familial Adenomatous Polyposis (FAP) and JNA's via the adenomatous polyposis coli (APC)/β-catenin pathway, but no specific genetic link has been identified as of yet.<ref name="Guertl 2000">Guertl B, Beham A, Zechner R, Stammberger H, Hoefler G. Nasopharyngeal angiofibroma: an AM-Gene-Associated tumor?. Human pathology. 2000 Nov 1;31(11):1411-3.</ref>

=== Histology ===
Grossly on histologic section there are large fibrous/collagenous sections with fibroblasts, as well as vascular spaces of various sizes throughout the tumor.<ref name="Xu 2020">Xu B. Nasopharyngeal angiofibroma. PathologyOutlines.com website. https://www.pathologyoutlines.com/topic/nasalangiofibroma.html. Accessed November 17th, 2024.</ref> The stromal cells have an abundance of beta-catenin relative to the vascular endothelial cells.

<gallery>
JNA 30x.png|H&E stained section of green inked vascular channel (30x)
Beta Catenin JNA.png|Abnormal nuclear expression in stromal cells (blue arrow), compared with membranous / cytoplasmic staining in the endothelial cells (red arrow)
</gallery>

== Diagnosis ==
=== Patient History ===
Patients may have a history of any of the following symptoms:
* Unilateral nasal obstruction
* Recurrent epistaxis
* High-volume epistaxis
* Eustachian tube dysfunction
* Headache
* Facial swelling
* Anosmia / Hyposmia
* Cranial neuropathy
* Vision changes

=== Physical Examination ===
Many patients will not have obvious findings on anterior rhinoscopy. A large vascular-appearing mass is commonly fond on nasal endoscopy on the posterior lateral nasal sidewall near the region of the sphenopalatine artery. Note that '''''these hypervascular masses should not be biopsied''''', as this may result in significant bleeding that is hard to control in the clinic setting.

=== Laboratory Tests ===
Laboratory tests are not commonly useful in the diagnosis of JNA's, but should be performed preoperatively in the case of a planned surgical resection.
* Hemoglobin / Hematocrit
* Platelet level
* Platelet function (if family history of platelet dysfunction)
* PT / INR
* Blood Type and Screen (all patients)
* Blood Type and Cross (patients with advanced stage tumors where a significant blood loss is expected)

=== Imaging ===
[[File:Holman-Miller Sign.png|right|thumb|300 px|Axial CT scan of a JNA showing a right-sided Holman Miller sign]]
CT scan, angiography, and MRI are all useful in the workup of JNA's and have differing benefits.<ref name="Gaillard 2008">Gaillard F, Walizai T, Bell D, et al. Juvenile nasopharyngeal angiofibroma. Reference article, Radiopaedia.org (Accessed on 17 Nov 2024) https://doi.org/10.53347/rID-1541</ref> CT is typically the first imaging modality ordered in these patients, and is helpful for delineating the extent of bony erosion. Angiography demonstrates the extent of the mass well, and is frequently used in pre-operative embolization 24-48 hours before surgical resection. A majority of JNA's are supplied by the external carotid system, either by the maxillary artery (SPA) or the ascending pharyngeal artery.<ref name="Gaillard 2008"></ref> A lesser fraction of tumors are supplied by the internal carotid system (ophthalmic or sphenoidal branches), and this is typically in larger masses with intracranial invasion. MRI can be used to assess for intraorbital or intracranial extension. JNA's are T1 intermediate, T2 heterogenous with flow voids, and enhance with Gadolinium. The following are general characteristics often found in imaging of JNA's:
* Highly vascular nasopharyngeal mass
* Holman-Miller Sign<ref name="Niknejad 2013">Niknejad M, Tatco V, Bell D, et al. Holman-Miller sign (maxillary sinus). Reference article, Radiopaedia.org (Accessed on 17 Nov 2024) https://doi.org/10.53347/rID-21804</ref>: Anterior displacement of the posterior maxillary wall due to mass effect from the tumor
* Widening of the PMF or PPF
* Erosion of the pterygoid plate, especially the medial plate

There are several classification systems that have been proposed for JNAs based on imaging findings.

{| class="wikitable", style="margin-left: auto; margin-right: auto; border: none; text-align: center"
|+ Classification Systems for Juvenile Nasopharyngeal Angiofibromas
|-
! Classification System !! style=width:15em | Stage I !! style=width:15em | Stage II !! style=width:15em | Stage III !! style=width:15em | Stage IV !! style=width:15em | Stage V
|-
| Andrews (1989)<ref name="Andrews 1989">Andrews JC, Fisch U, Aeppli U, Valavanis A, Makek MS. The surgical management of extensive nasopharyngeal angiofibromas with the infratemporal fossa approach. The Laryngoscope. 1989 Apr;99(4):429-37. https://doi.org/10.1288/00005537-198904000-00013</ref> || Confined to NP || Invading one of the following with evidence of bony erosion: PPF, MS, ES, or SS || Invading ITF or orbit '''IIIa''': No intracranial extension '''IIIb''': Extradural (parasellar) extension || Intradural extension '''IVa''': No infiltration of CS, PF, or OC '''IVb''': Infiltration of CS, PF, or OC || --
|-
| Chandler (1984)<ref name="Chandler 1984">Chandler JR, Moskowitz L, Goulding R, Quencer RM. Nasopharyngeal angiofibromas: staging and management. Annals of Otology, Rhinology & Laryngology. 1984 Jul;93(4):322-9. https://doi.org/10.1177/000348948409300408</ref> || Confined to NP || Extension into the nasal cavity, SS, or both || Extension into any of the following: antrum, ES, PMF, ITF, orbit, or cheek || Intracranial extension || --
|-
| Onerci (2006)<ref name="Onerci 2006">Onerci M, Oğretmenoğlu O, Yücel T. Juvenile nasopharyngeal angiofibroma: a revised staging system. Rhinology. 2006 Mar 1;44(1):39-45. PMID: 16550949</ref>|| Nose, NP, ES, and SS or minimal extension into PMF || MS involvement, full occupation of PMF, extension to anterior cranial fossa, limited extension into ITF || Deep extension into cancellous bone at pterygoid base or body and GW of sphenoid, significant lateral extension into ITF or pterygoid plates, orbital involvement, CS obliteration || Intracranial extension between pituitary gland and ICA, tumor localization lateral to ICA, middle fossa extension and extensive intracranial extension || --
|-
| Radkowski (1996)<ref name="Radkowski 1996">Radkowski D, McGill T, Healy GB, Ohlms L, Jones DT. Angiofibroma: changes in staging and treatment. Archives of Otolaryngology–Head & Neck Surgery. 1996 Feb 1;122(2):122-9. https://doi.org/10.1001/archotol.1996.01890140012004</ref>|| '''IA''': Confined to nose or NP '''IB''': Extends into one or more sinuses || '''IIa''': Minimal extension into medial PMF '''IIb''': Full occupation of PMF with local mass effect '''IIc''': Extension into ITF, cheek, or posterior to pterygoid plates || Erosion of skull base '''IIIa''': Minimal skull base involvement '''IIIb''': Extensive intracranial extension, with or without invasion into CS || -- || --
|-
| Sessions (1981)<ref name="Sessions 1981">Sessions RB, Bryan RN, Naclerio RM, Alford BR. Radiographic staging of juvenile angiofibroma. Head & neck surgery. 1981 Mar;3(4):279-83. https://doi.org/10.1002/hed.2890030404</ref> || '''IA''': Confined to nose or NP '''IB''': Extends into one or more sinuses || '''IIa''': Minimal extension into PMF '''IIb''': Full occupation of PMF with or without orbital erosion '''IIc''': ITF with or without cheek extension || Intracranial extension || -- || --
|-
| UPMC (2010)<ref name="Snyderman 2010">Snyderman CH, Pant H, Carrau RL, Gardner P. A new endoscopic staging system for angiofibromas. Archives of Otolaryngology–Head & Neck Surgery. 2010 Jun 21;136(6):588-94. https://doi.org/10.1001/archoto.2010.83</ref> || Nasal cavity, medial PPF || Paranasal sinuses, lateral PPF; no residual vascularity || Skull base erosion, orbit, ITF involvement; no residual vascularity || Skull base erosion, orbit, ITF involvement; residual vascularity || Intracranial extension with residual vascularity '''M''': Medial extension '''L''': Lateral extension
|-
|+ style="caption-side:bottom; font-weight: normal;"|''Abbreviations: Cavernous sinus (CS), Ethmoid sinus (ES), Infratemporal fossa (ITF), Internal carotid artery (ICA), Maxillary sinus (MS), Nasopharynx (NP), Optic chiasm (OC), Pituitary fossa (PF), Pterygomaxillary fissure (PMF), Pterygopalatine fossa (PPF), Sphenoid sinus (SS)''
|}

=== Differential Diagnosis ===
In the case of a nasopharyngeal mass, the following should also be considered:
* Clival chondroma
* Encephalocele
* Esthesioneuroblastoma
* Inverted papilloma
* Nasal polyp
* Nasopharyngeal carcinoma
* Nasopharyngeal teratoma
* Rhabdomyosarcoma
* Vascular malformation

== Management ==
=== Medical Management ===
There is no approved medical treatment for management of juvenile nasopharyngeal angiofibromas. The treatment is primarily surgical. Medical management focuses on optimizing patients before surgery, such as addressing any coagulopathies or addressing underlying anemia.

=== Surgical Management ===
Historically, surgical management of JNAs involved aggressive open approaches, including transpalatal, lateral rhinotomy, midface degloving, or infratemporal fossa craniotomy approaches.<ref name="Renkonen 2011">Renkonen S, Hagström J, Vuola J, Niemelä M, Porras M, Kivivuori SM, Leivo I, Mäkitie AA. The changing surgical management of juvenile nasopharyngeal angiofibroma. European archives of oto-rhino-laryngology. 2011 Apr;268:599-607. https://doi.org/10.1007/s00405-010-1383-z</ref> In recent years, there has been a transition towards transnasal endoscopic approaches. This has resulted in better visualization during surgery and a subsequent improvement in intraoperative blood loss, need for blood transfusions,<ref name="Oliveira 2012">Oliveira JA, Tavares MG, Aguiar CV, Azevedo JF, Sousa JR, Almeida PC, Gomes EF. Comparison between endoscopic and open surgery in 37 patients with nasopharyngeal angiofibroma. Brazilian Journal of otorhinolaryngology. 2012;78:75-80. https://doi.org/10.1590/S1808-86942012000100012</ref> and recurrence rates.<ref name="Bosraty 2011">Bosraty H, Atef A, Aziz M. Endoscopic vs. open surgery for treating large, locally advanced juvenile angiofibromas: A comparison of local control and morbidity outcomes. ENT: Ear, Nose & Throat Journal. 2011 Nov 1;90(11). PMID: 22109921</ref>

== Outcomes ==
=== Complications ===
=== Prognosis ===

== References ==
<references />

Juvenile Nasopharyngeal Angiofibroma

2024-11-17T21:09:20Z

Jack.Dewey:

{{infobox Disease
|Title =
|Aliases =
|Image = [[File:Nasopharyngeal angiofibroma - 2 - high mag.jpg]]
|Caption = Histologic section of a JNA
|ICD-9 = 210.7
|ICD-10 = D10.6
|MeSH =
|Gene =
|Locus =
|OMIM =
|EyeWiki =
|Radiopaedia = [https://radiopaedia.org/articles/juvenile-nasopharyngeal-angiofibroma?lang=us Juvenile Nasopharyngeal Angiofibroma]
|Pathology = [https://www.pathologyoutlines.com/topic/nasalangiofibroma.html Nasal Angiofibroma]
}}

== Overview ==
'''Juvenile nasopharyngeal angiofibroma (JNA)''' is a benign vascular neoplasm of the nasal cavity and nasopharynx that classically presents in adolescent boys.

== Pathophysiology ==
=== Relevant Anatomy ===
JNA's typically originate in the region of the sphenopalatine artery (SPA) in the posterior nasal cavity/nasopharynx. From there, they can extend into the paranasal sinuses, pterygopalatine fossa (PPF), infratemporal fossa (ITF), orbit, or cranial vault.

<gallery>
Pterygopalatine fossa.jpg|Cadaveric model of the PPF
Schematic diagram showing the bony anatomy and main neural connections of the PPF.png|Neural contents of the PPF
Gray511.png|Maxillary artery branches, with SPA at the distal end
Gerrish's Text-book of Anatomy (1902) - Fig. 243.png|Location of the sphenopalatine foramen
Basilar process and palatine process.jpg|Location of the ITF
</gallery>

=== Disease Etiology ===
JNA's are fibrovascular tumors that arise from the posterior lateral nasal cavity near the choana. There is substantial debate about the exact circumstances and location that lead to the formation of a JNA, but they are generally accepted to form near the region of the sphenopalatine foramen. Proposed theories regarding their etiology include androgen receptor-related growth,<ref name="Liu 2015">Liu Z, Wang J, Wang H, Wang D, Hu L, Liu Q, Sun X. Hormonal receptors and vascular endothelial growth factor in juvenile nasopharyngeal angiofibroma: immunohistochemical and tissue microarray analysis. Acta oto-laryngologica. 2015 Jan 2;135(1):51-7. https://doi.org/10.3109/00016489.2014.952774</ref> incomplete regression of a branchial artery during embryogenesis,<ref name="Schick 2002">Schick B, Plinkert PK, Prescher A. Aetiology of angiofibromas: reflection on their specific vascular component. Laryngo-rhino-otologie. 2002 Apr 1;81(4):280-4. https://doi.org/10.1055/s-2002-25322</ref> oncogenic mutations in C-MYC and C-KIT,<ref name="Pandey 2017">Pandey P, Mishra A, Tripathi AM, Verma V, Trivedi R, Singh HP, Kumar S, Patel B, Singh V, Pandey S, Pandey A. Current molecular profile of juvenile nasopharyngeal angiofibroma: first comprehensive study from India. The Laryngoscope. 2017 Mar;127(3):E100-6. https://doi.org/10.1002/lary.26250</ref> and a wide variety of other genetic aberrations.

===Epidemiology===
Juvenile nasopharyngeal angiofibroma has been reported to be the most common benign neoplasm of the nasopharynx in young males. It accounts for 0.05% to 0.5% of all head and neck tumors, with a reported incidence of 1 in 5,000 to 1 in 60,000 in the US annually.<ref name="Boghani 2013">Boghani Z, Husain Q, Kanumuri VV, Khan MN, Sangvhi S, Liu JK, Eloy JA. Juvenile nasopharyngeal angiofibroma: a systematic review and comparison of endoscopic, endoscopic‐assisted, and open resection in 1047 cases. The Laryngoscope. 2013 Apr;123(4):859-69. https://doi.org/10.1002/lary.23843</ref><ref name="Ferouz 1995">Ferouz AS, Mohr RM, Paul P. Juvenile nasopharyngeal angiofibroma and familial adenomatous polyposis: an association?. Otolaryngology—Head and Neck Surgery. 1995 Oct;113(4):435-9. https://doi.org/10.1016/S0194-59989570081-1</ref><ref name="Huang 2014">Huang Y, Liu Z, Wang J, Sun X, Yang L, Wang D. Surgical management of juvenile nasopharyngeal angiofibroma: analysis of 162 cases from 1995 to 2012. The Laryngoscope. 2014 Aug;124(8):1942-6. https://doi.org/10.1002/lary.24522</ref> It classically presents in adolescent males, with a typical age of presentation between 13 and 22 years old.<ref name ="Newman 2023">Newman M, Nguyen TB, McHugh T, Reddy K, Sommer DD. Early-onset juvenile nasopharyngeal angiofibroma (JNA): a systematic review. Journal of Otolaryngology-Head & Neck Surgery. 2023 Dec;52(1):s40463-023. https://doi.org/10.1186/s40463-023-00687-w</ref>

=== Genetics ===
There is no identified definitive genetic locus that predisposes patients to JNA's. However, there have been a number of studies looking at the various genetic components that could influence their formation.<ref name="Doody 2019">Doody J, Adil EA, Trenor III CC, Cunningham MJ. The genetic and molecular determinants of juvenile nasopharyngeal angiofibroma: a systematic review. Annals of Otology, Rhinology & Laryngology. 2019 Nov;128(11):1061-72. https://doi.org/10.1177/0003489419850194</ref> Due to fact that JNA's classically form in adolescent males, there have been several studies focused on the possible hormonal influence on their formation and growth. JNA's tend to have an abundance of progesterone and estradiol, but a relatively lower concentration of testosterone and dihydrotestosterone.<ref name="Kumagami 05 1993">Kumagami H. Estradiol, dihydrotestosterone, and testosterone in juvenile nasopharyngeal angiofibroma tissue. American Journal of Rhinology. 1993 May;7(3):101-4. https://doi.org/10.2500/105065893781976393</ref><ref name="Kumagami 01 1993">Kumagami H. Sex hormones in juvenile nasopharyngeal angiofibroma tissue. Auris Nasus Larynx. 1993 Jan 1;20(2):131-5. https://doi.org/10.1016/S0385-8146(12)80240-9</ref> Supplemental testosterone was trialed in JNA patients, but both tumor growth<ref name="Johnsen 1966">Johnsen S, Kloster JH, Schiff M. The action of hormones on juvenile nasopharyngeal angiofibroma. Acta Oto-Laryngologica. 1966 Jan 1;61(1-6):153-60. https://doi.org/10.3109/00016486609127052</ref> and tumor recurrence<ref name="Riggs 2010">Riggs S, Orlandi RR. Juvenile nasopharyngeal angiofibroma recurrence associated with exogenous testosterone therapy. Head & Neck: Journal for the Sciences and Specialties of the Head and Neck. 2010 Jun;32(6):812-5. https://doi.org/10.1002/hed.21152</ref> have been documented. Papers investigating potential targeted therapy markers have looked at steroid hormones, chromosomal abnormalities, growth factors, and other intracellular molecular targets.<ref name="Doody 2019"></ref> There have been studies investigating a possible link between Familial Adenomatous Polyposis (FAP) and JNA's via the adenomatous polyposis coli (APC)/β-catenin pathway, but no specific genetic link has been identified as of yet.<ref name="Guertl 2000">Guertl B, Beham A, Zechner R, Stammberger H, Hoefler G. Nasopharyngeal angiofibroma: an AM-Gene-Associated tumor?. Human pathology. 2000 Nov 1;31(11):1411-3.</ref>

=== Histology ===
Grossly on histologic section there are large fibrous/collagenous sections with fibroblasts, as well as vascular spaces of various sizes throughout the tumor.<ref name="Xu 2020">Xu B. Nasopharyngeal angiofibroma. PathologyOutlines.com website. https://www.pathologyoutlines.com/topic/nasalangiofibroma.html. Accessed November 17th, 2024.</ref> The stromal cells have an abundance of beta-catenin relative to the vascular endothelial cells.

<gallery>
JNA 30x.png|H&E stained section of green inked vascular channel (30x)
Beta Catenin JNA.png|Abnormal nuclear expression in stromal cells (blue arrow), compared with membranous / cytoplasmic staining in the endothelial cells (red arrow)
</gallery>

== Diagnosis ==
=== Patient History ===
Patients may have a history of any of the following symptoms:
* Unilateral nasal obstruction
* Recurrent epistaxis
* High-volume epistaxis
* Eustachian tube dysfunction
* Headache
* Facial swelling
* Anosmia / Hyposmia
* Cranial neuropathy
* Vision changes

=== Physical Examination ===
Many patients will not have obvious findings on anterior rhinoscopy. A large vascular-appearing mass is commonly fond on nasal endoscopy on the posterior lateral nasal sidewall near the region of the sphenopalatine artery. Note that '''''these hypervascular masses should not be biopsied''''', as this may result in significant bleeding that is hard to control in the clinic setting.

=== Laboratory Tests ===
Laboratory tests are not commonly useful in the diagnosis of JNA's, but should be performed preoperatively in the case of a planned surgical resection.
* Hemoglobin / Hematocrit
* Platelet level
* Platelet function (if family history of platelet dysfunction)
* PT / INR
* Blood Type and Screen (all patients)
* Blood Type and Cross (patients with advanced stage tumors where a significant blood loss is expected)

=== Imaging ===
[[File:Holman-Miller Sign.png|right|thumb|300 px|Axial CT scan of a JNA showing a right-sided Holman Miller sign]]
CT scan, angiography, and MRI are all useful in the workup of JNA's and have differing benefits.<ref name="Gaillard 2008">Gaillard F, Walizai T, Bell D, et al. Juvenile nasopharyngeal angiofibroma. Reference article, Radiopaedia.org (Accessed on 17 Nov 2024) https://doi.org/10.53347/rID-1541</ref> CT is typically the first imaging modality ordered in these patients, and is helpful for delineating the extent of bony erosion. Angiography demonstrates the extent of the mass well, and is frequently used in pre-operative embolization 24-48 hours before surgical resection. A majority of JNA's are supplied by the external carotid system, either by the maxillary artery (SPA) or the ascending pharyngeal artery.<ref name="Gaillard 2008"></ref> A lesser fraction of tumors are supplied by the internal carotid system (ophthalmic or sphenoidal branches), and this is typically in larger masses with intracranial invasion. MRI can be used to assess for intraorbital or intracranial extension. JNA's are T1 intermediate, T2 heterogenous with flow voids, and enhance with Gadolinium. The following are general characteristics often found in imaging of JNA's:
* Highly vascular nasopharyngeal mass
* Holman-Miller Sign<ref name="Niknejad 2013">Niknejad M, Tatco V, Bell D, et al. Holman-Miller sign (maxillary sinus). Reference article, Radiopaedia.org (Accessed on 17 Nov 2024) https://doi.org/10.53347/rID-21804</ref>: Anterior displacement of the posterior maxillary wall due to mass effect from the tumor
* Widening of the PMF or PPF
* Erosion of the pterygoid plate, especially the medial plate

There are several classification systems that have been proposed for JNAs based on imaging findings.

{| class="wikitable", style="margin-left: auto; margin-right: auto; border: none; text-align: center"
|+ Classification Systems for Juvenile Nasopharyngeal Angiofibromas
|-
! Classification System !! style=width:15em | Stage I !! style=width:15em | Stage II !! style=width:15em | Stage III !! style=width:15em | Stage IV !! style=width:15em | Stage V
|-
| Andrews (1989)<ref name="Andrews 1989">Andrews JC, Fisch U, Aeppli U, Valavanis A, Makek MS. The surgical management of extensive nasopharyngeal angiofibromas with the infratemporal fossa approach. The Laryngoscope. 1989 Apr;99(4):429-37. https://doi.org/10.1288/00005537-198904000-00013</ref> || Confined to NP || Invading one of the following with evidence of bony erosion: PPF, MS, ES, or SS || Invading ITF or orbit '''IIIa''': No intracranial extension '''IIIb''': Extradural (parasellar) extension || Intradural extension '''IVa''': No infiltration of CS, PF, or OC '''IVb''': Infiltration of CS, PF, or OC || --
|-
| Chandler (1984)<ref name="Chandler 1984">Chandler JR, Moskowitz L, Goulding R, Quencer RM. Nasopharyngeal angiofibromas: staging and management. Annals of Otology, Rhinology & Laryngology. 1984 Jul;93(4):322-9. https://doi.org/10.1177/000348948409300408</ref> || Confined to NP || Extension into the nasal cavity, SS, or both || Extension into any of the following: antrum, ES, PMF, ITF, orbit, or cheek || Intracranial extension || --
|-
| Onerci (2006)<ref name="Onerci 2006">Onerci M, Oğretmenoğlu O, Yücel T. Juvenile nasopharyngeal angiofibroma: a revised staging system. Rhinology. 2006 Mar 1;44(1):39-45. PMID: 16550949</ref>|| Nose, NP, ES, and SS or minimal extension into PMF || MS involvement, full occupation of PMF, extension to anterior cranial fossa, limited extension into ITF || Deep extension into cancellous bone at pterygoid base or body and GW of sphenoid, significant lateral extension into ITF or pterygoid plates, orbital involvement, CS obliteration || Intracranial extension between pituitary gland and ICA, tumor localization lateral to ICA, middle fossa extension and extensive intracranial extension || --
|-
| Radkowski (1996)<ref name="Radkowski 1996">Radkowski D, McGill T, Healy GB, Ohlms L, Jones DT. Angiofibroma: changes in staging and treatment. Archives of Otolaryngology–Head & Neck Surgery. 1996 Feb 1;122(2):122-9. https://doi.org/10.1001/archotol.1996.01890140012004</ref>|| '''IA''': Confined to nose or NP '''IB''': Extends into one or more sinuses || '''IIa''': Minimal extension into medial PMF '''IIb''': Full occupation of PMF with local mass effect '''IIc''': Extension into ITF, cheek, or posterior to pterygoid plates || Erosion of skull base '''IIIa''': Minimal skull base involvement '''IIIb''': Extensive intracranial extension, with or without invasion into CS || -- || --
|-
| Sessions (1981)<ref name="Sessions 1981">Sessions RB, Bryan RN, Naclerio RM, Alford BR. Radiographic staging of juvenile angiofibroma. Head & neck surgery. 1981 Mar;3(4):279-83. https://doi.org/10.1002/hed.2890030404</ref> || '''IA''': Confined to nose or NP '''IB''': Extends into one or more sinuses || '''IIa''': Minimal extension into PMF '''IIb''': Full occupation of PMF with or without orbital erosion '''IIc''': ITF with or without cheek extension || Intracranial extension || -- || --
|-
| UPMC (2010)<ref name="Snyderman 2010">Snyderman CH, Pant H, Carrau RL, Gardner P. A new endoscopic staging system for angiofibromas. Archives of Otolaryngology–Head & Neck Surgery. 2010 Jun 21;136(6):588-94. https://doi.org/10.1001/archoto.2010.83</ref> || Nasal cavity, medial PPF || Paranasal sinuses, lateral PPF; no residual vascularity || Skull base erosion, orbit, ITF involvement; no residual vascularity || Skull base erosion, orbit, ITF involvement; residual vascularity || Intracranial extension with residual vascularity '''M''': Medial extension '''L''': Lateral extension
|-
|+ style="caption-side:bottom; font-weight: normal;"|''Abbreviations: Cavernous sinus (CS), Ethmoid sinus (ES), Infratemporal fossa (ITF), Internal carotid artery (ICA), Maxillary sinus (MS), Nasopharynx (NP), Optic chiasm (OC), Pituitary fossa (PF), Pterygomaxillary fissure (PMF), Pterygopalatine fossa (PPF), Sphenoid sinus (SS)''
|}

=== Differential Diagnosis ===
In the case of a nasopharyngeal mass, the following should also be considered:
* Clival chondroma
* Encephalocele
* Esthesioneuroblastoma
* Inverted papilloma
* Nasal polyp
* Nasopharyngeal carcinoma
* Nasopharyngeal teratoma
* Rhabdomyosarcoma
* Vascular malformation

== Management ==
=== Medical Management ===
There is no approved medical treatment for management of juvenile nasopharyngeal angiofibromas. The treatment is primarily surgical. Medical management focuses on optimizing patients before surgery, such as addressing any coagulopathies or addressing underlying anemia.

=== Surgical Management ===

== Outcomes ==
=== Complications ===
=== Prognosis ===

== References ==
<references />