Juvenile Nasopharyngeal 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.
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Cadaveric model of the PPF
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Neural contents of the PPF
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Maxillary artery branches, with SPA at the distal end
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Location of the sphenopalatine foramen
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Location of the ITF
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Disease Etiology
Genetics
Histology
Diagnosis
Patient History
Physical Examination
Laboratory Tests
Imaging
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There are several classification systems that have been proposed for JNAs based on imaging findings.
| Classification System | Stage I | Stage II | Stage III | Stage IV | Stage V |
|---|---|---|---|---|---|
| Andrews (1989)[1] | 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)[2] | 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)[3] | 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)[4] | 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)[5] | 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)[6] | 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 |
Differential Diagnosis
Management
Medical Management
Surgical Management
Outcomes
Complications
Prognosis
References
- ↑ 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.
- ↑ Chandler JR, Moskowitz L, Goulding R, Quencer RM. Nasopharyngeal angiofibromas: staging and management. Annals of Otology, Rhinology & Laryngology. 1984 Jul;93(4):322-9.
- ↑ Onerci M, Oğretmenoğlu O, Yücel T. Juvenile nasopharyngeal angiofibroma: a revised staging system. Rhinology. 2006 Mar 1;44(1):39-45.
- ↑ 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.
- ↑ Sessions RB, Bryan RN, Naclerio RM, Alford BR. Radiographic staging of juvenile angiofibroma. Head & neck surgery. 1981 Mar;3(4):279-83.
- ↑ 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.