Paraganglioma - Revision history

Jack.Dewey: /* Management */

2024-09-15T22:43:56Z

Management

← Older revision		Revision as of 22:43, 15 September 2024
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	For carotid body paragangliomas, patients with a Shamblin Type I or Type II tumor are more amenable to surgical resection.<ref>Lim JY, Kim J, Kim SH, Lee S, Lim YC, Kim JW, Choi EC. Surgical treatment of carotid body paragangliomas: outcomes and complications according to the shamblin classification. Clinical and experimental otorhinolaryngology. 2010 Jun 30;3(2):91-5.</ref> Patients with a Shamblin Type III carotid body mass may require arterial bypass and resection, for which vascular surgery should be involved.		For carotid body paragangliomas, patients with a Shamblin Type I or Type II tumor are more amenable to surgical resection.<ref>Lim JY, Kim J, Kim SH, Lee S, Lim YC, Kim JW, Choi EC. Surgical treatment of carotid body paragangliomas: outcomes and complications according to the shamblin classification. Clinical and experimental otorhinolaryngology. 2010 Jun 30;3(2):91-5.</ref> Patients with a Shamblin Type III carotid body mass may require arterial bypass and resection, for which vascular surgery should be involved.

	~~Jugulotympanic~~ paragangliomas		Traditionally, jugulotympanic paragangliomas were managed surgically with an infratemporal fossa approach. This is associated with significant postoperative morbidity including hearing loss and facial paralysis. Conservative measures are more favorable where possible for these patients. If there is intracranial extension of the mass, this often requires a combined approach with surgical removal of the middle ear and mastoid components and intracranial radiation.

	== Outcomes ==		== Outcomes ==

Jack.Dewey: /* Disease Etiology */

2024-09-15T22:39:26Z

Disease Etiology

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	Paragangliomas are a result of unregulated growth of paragangliar tissue. A majority of these masses arise from the carotid body just superior to the carotid bifurcation. Paragangliomas that arise from the area around the temporal bone are termed ''jugulotympanic'' paragangliomas. The term ''glomus tympanicum'' historically describes paragangliomas that arise from Jacobson's nerve (the tympanic branch of CN XI) - by definition these arise on the cochlea promontory and are confined to the middle ear and mastoid cavity. The term ''glomus jugulare'' was previously used to describe paragangliomas that arise along Jacobson's nerve or Arnold's nerve (the auricular branch of CN X), and involve the jugular bulb or skull base. These two terms have fallen out of favor and are more commonly described now collectively as "jugulotympanic" paragangliomas.		Paragangliomas are a result of unregulated growth of paragangliar tissue. A majority of these masses arise from the carotid body just superior to the carotid bifurcation. Paragangliomas that arise from the area around the temporal bone are termed ''jugulotympanic'' paragangliomas. The term ''glomus tympanicum'' historically describes paragangliomas that arise from Jacobson's nerve (the tympanic branch of CN XI) - by definition these arise on the cochlea promontory and are confined to the middle ear and mastoid cavity. The term ''glomus jugulare'' was previously used to describe paragangliomas that arise along Jacobson's nerve or Arnold's nerve (the auricular branch of CN X), and involve the jugular bulb or skull base. These two terms have fallen out of favor and are more commonly described now collectively as "jugulotympanic" paragangliomas.

	In rare instances these masses have been documented to metastasize, in what is termed a "malignant paraganglioma". Typical metastases are to the cervical lymph nodes but distant metastases to the bone, liver, and lung have also been described.		In rare instances these masses have been documented to metastasize, in what is termed a "malignant paraganglioma". Typical metastases are to the cervical lymph nodes but distant metastases to the bone, liver, and lung have also been described. Of note, syndromic paragangliomas are less often malignant compared to sporadic
			disease.

	=== Epidemiology ===		=== Epidemiology ===

Jack.Dewey at 22:37, 15 September 2024

2024-09-15T22:37:04Z

← Older revision		Revision as of 22:37, 15 September 2024
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	=== Disease Etiology ===		=== Disease Etiology ===
			Paragangliomas are a result of unregulated growth of paragangliar tissue. A majority of these masses arise from the carotid body just superior to the carotid bifurcation. Paragangliomas that arise from the area around the temporal bone are termed ''jugulotympanic'' paragangliomas. The term ''glomus tympanicum'' historically describes paragangliomas that arise from Jacobson's nerve (the tympanic branch of CN XI) - by definition these arise on the cochlea promontory and are confined to the middle ear and mastoid cavity. The term ''glomus jugulare'' was previously used to describe paragangliomas that arise along Jacobson's nerve or Arnold's nerve (the auricular branch of CN X), and involve the jugular bulb or skull base. These two terms have fallen out of favor and are more commonly described now collectively as "jugulotympanic" paragangliomas.

			In rare instances these masses have been documented to metastasize, in what is termed a "malignant paraganglioma". Typical metastases are to the cervical lymph nodes but distant metastases to the bone, liver, and lung have also been described.

	=== Epidemiology ===		=== Epidemiology ===

Jack.Dewey at 22:18, 15 September 2024

2024-09-15T22:18:47Z

← Older revision		Revision as of 22:18, 15 September 2024
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	! Type I !! Type II !! Type III		! Type I !! Type II !! Type III
	\|-		\|-
	\| ~~Example~~ \|\| ~~Example~~ \|\| ~~Example~~		\|[[image:Shamblin 1 Cross Section.jpg \| center \| 300px ]] \|\| [[image:Shamblin 2 Cross Section.jpg \| center \| 300px ]] \|\| [[image:Shamblin 3 Cross Section.jpg \| center \| 300px ]]
	\|-		\|-
	\| Relatively small with minimal attachment to the carotid vessels \|\| Somewhat larger with more extensive attachment to the carotid vessels \|\| Large mass with complete encasement of the carotid vessels		\| Relatively small with minimal attachment to the carotid vessels \|\| Somewhat larger with more extensive attachment to the carotid vessels \|\| Large mass with complete encasement of the carotid vessels

Jack.Dewey: /* Genetics */

2024-09-15T21:42:02Z

Genetics

← Older revision		Revision as of 21:42, 15 September 2024
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	=== Genetics ===		=== Genetics ===
	There are several genetic syndromes associated with paragangliomas of the head and neck.		There are several genetic syndromes associated with paragangliomas of the head and neck.<ref>Tsirlin A, Oo Y, Sharma R, Kansara A, Gliwa A, Banerji MA. Pheochromocytoma: a review. Maturitas. 2014 Mar 1;77(3):229-38.</ref>

	{\| class="wikitable sortable", style="margin-left: auto; margin-right: auto; border: none; text-align: center"		{\| class="wikitable sortable", style="margin-left: auto; margin-right: auto; border: none; text-align: center"

Jack.Dewey at 21:40, 15 September 2024

2024-09-15T21:40:44Z

← Older revision		Revision as of 21:40, 15 September 2024
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	\|ICD-10 = Carotid: D44.6, Jugular: D44.7		\|ICD-10 = Carotid: D44.6, Jugular: D44.7
	\|MeSH =		\|MeSH =
	\|Gene =		\|Gene = [https://www.ncbi.nlm.nih.gov/gene/6392 ''SDHD''], [https://www.ncbi.nlm.nih.gov/gene/54949 ''SDHAF2''], [https://www.ncbi.nlm.nih.gov/gene/6391 ''SDHC''], [https://www.ncbi.nlm.nih.gov/gene/6390 ''SDHB''], [https://www.ncbi.nlm.nih.gov/gene/6389 ''SDHA''], [https://www.ncbi.nlm.nih.gov/gene/7428 ''VHL''], [https://www.ncbi.nlm.nih.gov/gene/5979 ''RET''], [https://www.ncbi.nlm.nih.gov/gene/4763 ''NF1'']
	\|Locus =		\|Locus = 11q23.1, 11q13.1, 1q23.3, 1p36.13, 5p15.33, 3p25.3, 10q11.21, 17q11.2 (respectively with above)
	\|OMIM =		\|OMIM =
	\|EyeWiki =		\|EyeWiki =
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	! Syndrome !! Gene !! Locus !! Inheritance !! Additional Findings		! Syndrome !! Gene !! Locus !! Inheritance !! Additional Findings
	\|-		\|-
	\| Paraganglioma Syndrome Type 1 (PGL1) \|\| ''SDHD'' \|\| 11q23 \|\| AD, Maternal Imprinting \|\| Pituitary adenoma, GIST, RCC		\| Paraganglioma Syndrome Type 1 (PGL1) \|\| [https://www.ncbi.nlm.nih.gov/gene/6392 ''SDHD''] \|\| 11q23.1 \|\| AD, Maternal Imprinting \|\| Pituitary adenoma, GIST, RCC
	\|-		\|-
	\| Paraganglioma Syndrome Type 2 (PGL2) \|\| ''SDHAF2'' \|\| 11q13.1 \|\| AD, Maternal Imprinting \|\| Pituitary adenoma, GIST, RCC		\| Paraganglioma Syndrome Type 2 (PGL2) \|\| [https://www.ncbi.nlm.nih.gov/gene/54949 ''SDHAF2''] \|\| 11q13.1 \|\| AD, Maternal Imprinting \|\| Pituitary adenoma, GIST, RCC
	\|-		\|-
	\| Paraganglioma Syndrome Type 3 (PGL3) \|\| ''SDHC'' \|\| ~~1q21~~ \|\| AD \|\| Pituitary adenoma, GIST, RCC		\| Paraganglioma Syndrome Type 3 (PGL3) \|\| [https://www.ncbi.nlm.nih.gov/gene/6391 ''SDHC''] \|\| 1q23.3 \|\| AD \|\| Pituitary adenoma, GIST, RCC
	\|-		\|-
	\| Paraganglioma Syndrome Type 4 (PGL4) \|\| ''SDHB'' \|\| ~~1p35-36~~ \|\| AD \|\| Pituitary adenoma, GIST, RCC		\| Paraganglioma Syndrome Type 4 (PGL4) \|\| [https://www.ncbi.nlm.nih.gov/gene/6390 ''SDHB''] \|\| 1p36.13 \|\| AD \|\| Pituitary adenoma, GIST, RCC
	\|-		\|-
	\| Paraganglioma Syndrome Type 5 (PGL5) \|\| ''SDHA'' \|\| 5p15.33 \|\| AD \|\| Pituitary adenoma, GIST, RCC		\| Paraganglioma Syndrome Type 5 (PGL5) \|\| [https://www.ncbi.nlm.nih.gov/gene/6389 ''SDHA''] \|\| 5p15.33 \|\| AD \|\| Pituitary adenoma, GIST, RCC
	\|-		\|-
	\| von Hippel-Lindau Syndrome \|\| ''VHL'' \|\| 3p25~~-26~~ \|\| AD \|\| Hemangioblastoma, retinal angioma, clear cell RCC, pancreatic neuroendocrine tumors, endolymphatic sac tumors		\| von Hippel-Lindau Syndrome \|\| [https://www.ncbi.nlm.nih.gov/gene/7428 ''VHL''] \|\| 3p25.3 \|\| AD \|\| Hemangioblastoma, retinal angioma, clear cell RCC, pancreatic neuroendocrine tumors, endolymphatic sac tumors
	\|-		\|-
	\| Multiple Endocrine Neoplasia type 2 (MEN-2) \|\| ''RET'' \|\| 10q11.21 \|\| AD \|\| Medullary thyroid carcinoma, Hyperparathyroidism		\| Multiple Endocrine Neoplasia type 2 (MEN-2) \|\| [https://www.ncbi.nlm.nih.gov/gene/5979 ''RET''] \|\| 10q11.21 \|\| AD \|\| Medullary thyroid carcinoma, Hyperparathyroidism
	\|-		\|-
	\| Neurofibromatosis Type 1 (NF1) \|\| ''NF1'' \|\| 17q11.2 \|\| AD \|\| Neurofibromas, cafe-au-lait spots, cognitive deficits, Lisch nodules		\| Neurofibromatosis Type 1 (NF1) \|\| [https://www.ncbi.nlm.nih.gov/gene/4763 ''NF1''] \|\| 17q11.2 \|\| AD \|\| Neurofibromas, cafe-au-lait spots, cognitive deficits, Lisch nodules
	\|}		\|}

Jack.Dewey: /* Genetics */

2024-09-15T21:27:01Z

Genetics

← Older revision		Revision as of 21:27, 15 September 2024
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	\| Paraganglioma Syndrome Type 5 (PGL5) \|\| ''SDHA'' \|\| 5p15.33 \|\| AD \|\| Pituitary adenoma, GIST, RCC		\| Paraganglioma Syndrome Type 5 (PGL5) \|\| ''SDHA'' \|\| 5p15.33 \|\| AD \|\| Pituitary adenoma, GIST, RCC
	\|-		\|-
	\| ~~Example~~ \|\| ~~Example~~ \|\| ~~Example~~ \|\| ~~Example~~ \|\| ~~Example~~		\| von Hippel-Lindau Syndrome \|\| ''VHL'' \|\| 3p25-26 \|\| AD \|\| Hemangioblastoma, retinal angioma, clear cell RCC, pancreatic neuroendocrine tumors, endolymphatic sac tumors
			\|-
			\| Multiple Endocrine Neoplasia type 2 (MEN-2) \|\| ''RET'' \|\| 10q11.21 \|\| AD \|\| Medullary thyroid carcinoma, Hyperparathyroidism
			\|-
			\| Neurofibromatosis Type 1 (NF1) \|\| ''NF1'' \|\| 17q11.2 \|\| AD \|\| Neurofibromas, cafe-au-lait spots, cognitive deficits, Lisch nodules
	\|}		\|}

Jack.Dewey at 21:19, 15 September 2024

2024-09-15T21:19:41Z

← Older revision		Revision as of 21:19, 15 September 2024
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	There are several genetic syndromes associated with paragangliomas of the head and neck.		There are several genetic syndromes associated with paragangliomas of the head and neck.

	{\| class="wikitable sortable"		{\| class="wikitable sortable", style="margin-left: auto; margin-right: auto; border: none; text-align: center"
	\|+ Genetic Syndromes Associated with Head and Neck Paragangliomas		\|+ Genetic Syndromes Associated with Head and Neck Paragangliomas
	\|-		\|-
	! ~~Header text~~ !! ~~Header text~~ !! ~~Header text~~ !! ~~Header text~~ !! ~~Header text~~		! Syndrome !! Gene !! Locus !! Inheritance !! Additional Findings
	\|-		\|-
	\| ~~Example~~ \|\| ~~Example~~ \|\| ~~Example~~ \|\| ~~Example~~ \|\| ~~Example~~		\| Paraganglioma Syndrome Type 1 (PGL1) \|\| ''SDHD'' \|\| 11q23 \|\| AD, Maternal Imprinting \|\| Pituitary adenoma, GIST, RCC
	\|-		\|-
	\| ~~Example~~ \|\| ~~Example~~ \|\| ~~Example~~ \|\| ~~Example~~ \|\| ~~Example~~		\| Paraganglioma Syndrome Type 2 (PGL2) \|\| ''SDHAF2'' \|\| 11q13.1 \|\| AD, Maternal Imprinting \|\| Pituitary adenoma, GIST, RCC
	\|-		\|-
	\| ~~Example~~ \|\| ~~Example~~ \|\| ~~Example~~ \|\| ~~Example~~ \|\| ~~Example~~		\| Paraganglioma Syndrome Type 3 (PGL3) \|\| ''SDHC'' \|\| 1q21 \|\| AD \|\| Pituitary adenoma, GIST, RCC
	\|-		\|-
	\| ~~Example~~ \|\| ~~Example~~ \|\| ~~Example~~ \|\| ~~Example~~ \|\| ~~Example~~		\| Paraganglioma Syndrome Type 4 (PGL4) \|\| ''SDHB'' \|\| 1p35-36 \|\| AD \|\| Pituitary adenoma, GIST, RCC
	\|-		\|-
	\| ~~Example~~ \|\| ~~Example~~ \|\| ~~Example~~ \|\| ~~Example~~ \|\| ~~Example~~		\| Paraganglioma Syndrome Type 5 (PGL5) \|\| ''SDHA'' \|\| 5p15.33 \|\| AD \|\| Pituitary adenoma, GIST, RCC
	\|-		\|-
	\| Example \|\| Example \|\| Example \|\| Example \|\| Example		\| Example \|\| Example \|\| Example \|\| Example \|\| Example

Jack.Dewey: /* Genetics */

2024-09-15T21:11:06Z

Genetics

← Older revision		Revision as of 21:11, 15 September 2024
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	=== Genetics ===		=== Genetics ===
			There are several genetic syndromes associated with paragangliomas of the head and neck.

			{\| class="wikitable sortable"
			\|+ Genetic Syndromes Associated with Head and Neck Paragangliomas
			\|-
			! Header text !! Header text !! Header text !! Header text !! Header text
			\|-
			\| Example \|\| Example \|\| Example \|\| Example \|\| Example
			\|-
			\| Example \|\| Example \|\| Example \|\| Example \|\| Example
			\|-
			\| Example \|\| Example \|\| Example \|\| Example \|\| Example
			\|-
			\| Example \|\| Example \|\| Example \|\| Example \|\| Example
			\|-
			\| Example \|\| Example \|\| Example \|\| Example \|\| Example
			\|-
			\| Example \|\| Example \|\| Example \|\| Example \|\| Example
			\|}

	=== Histology ===		=== Histology ===

Jack.Dewey: /* Differential Diagnosis */

2024-09-15T21:03:14Z

Differential Diagnosis

← Older revision		Revision as of 21:03, 15 September 2024
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	\|}		\|}

	In addition, the following diagnoses should be considered:		There are several paraganglioma-associated syndromes that should be considered if the patient has any of the associated characteristic findings of each:
			* von Hippel-Lindau (VHL) Syndrome
			* Multiple Endocrine Neoplasia type 2 (MEN-2)
			* Neurofibromatosis type 1 (NF1)
			* Succinate Dehydrogenase-associated paraganglioma syndromes

			In addition, the following diagnoses should be considered for patients with new neck masses:
	* Schwannoma		* Schwannoma
	* Arterio-Venous Malformation		* Arterio-Venous Malformation