Paraganglioma

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Paraganglioma
ICD-O:	8680-8700
DiseasesDB	33480

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

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.

Medical Therapy

The main treatment modalities are surgery, embolization and radiotherapy.