Paraganglioma: Difference between revisions

	Paraganglioma;
ICD-O:	8680-8700
DiseasesDB	33480

	Paraganglioma Microchapters
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Revision as of 18:59, 20 January 2012

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Editor-In-Chief: C. Michael Gibson, M.S., M.D. [1]

Overview

Inheritance

Familial paragangliomas account for approx. 25% of cases, are often multiple and bilateral, and occur at an earlier age. Mutations of the genes SDHD (previously known as PGL1), PGL2, and SDHC (previously PGL3) have been identified as causing familial head and neck paragangliomas. Mutations of SDHB play an important role in familial adrenal pheochromocytoma and extra-adrenal paraganglioma (of abdomen and thorax), although there is considerable overlap in the types of tumors associated with SDHB and SDHD gene mutations.

References

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 ==[[Paraganglioma history and symptoms|History & Symptoms]]==
-Paragangliomas are described by their site of origin and are often given special names:
-* '''Carotid paraganglioma''' ([[carotid body]] tumor): Is the most common of the head and neck paragangliomas.  It usually presents as a painless neck mass, but larger tumors may cause cranial nerve palsies, usually of the [[vagus nerve]] and [[hypoglossal nerve]].
-* '''Glomus tympanicum''' and '''Glomus jugulare''': Both commonly present as a middle ear mass resulting in [[tinnitus]] (in 80%) and hearing loss (in 60%).  The cranial nerves of the [[jugular foramen]] may be compressed, resulting swallowing difficulty.  These patients present with a reddish bulge behind an intact ear drum.  This condition is also known as the "Red drum".  On application of pressure to the external ear canal with the help of a pneumatic ear speculum the mass could be seen to blanch.  This sign is known as "Brown's sign".
-* '''Vagal paragangliomas''': These are the least common of the head and neck paragangliomas.  They usually present as a painless neck mass, but may result in [[dysphagia]] and hoarseness.
-* '''Other sites''': Rare sites of involvement are the [[larynx]], nasal cavity, paranasal sinuses, [[thyroid]] gland, and the [[thoracic inlet]], as well as the bladder in extremely rare cases.
 ==[[Paraganglioma medical therapy|Medical Therapy]]==

Paraganglioma: Difference between revisions

Revision as of 18:59, 20 January 2012

Contents

Overview

Inheritance

Pathophysiology

History & Symptoms

Medical Therapy

References

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