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VOLUME 16 , ISSUE 1 ( Jan-Jun, 2021 ) > List of Articles

REVIEW ARTICLE

Succinate Dehydrogenase Mutation and Paraganglioma Syndromes: A Review Article

Surabhi Garg, Loreno Enny, Upander Kumar, Nancy Raja, Pooja Ramakant, Kul Ranjan Singh, Anand Mishra

Keywords : Paraganglioma, Pheochromocytoma, Succinate dehydrogenase, Endocrine surgery

Citation Information : Garg S, Enny L, Kumar U, Raja N, Ramakant P, Singh KR, Mishra A. Succinate Dehydrogenase Mutation and Paraganglioma Syndromes: A Review Article. 2021; 16 (1):11-16.

DOI: 10.5005/jp-journals-10088-11162

License: CC BY-NC 4.0

Published Online: 26-08-2021

Copyright Statement:  Copyright © 2021; Jaypee Brothers Medical Publishers (P) Ltd.


Abstract

Pheochromocytomas and paragangliomas are rare tumours of both sympathetic and parasympathetic origin. Pheochromocytomas are derived from the adrenal medulla whereas paraganglioma arise from extra adrenal sympathetic and parasympathetic tissues. Between a quarter to one-third of pheochromocytomas-paragangliomas have familial aetiology which are heterogenous and include syndromes like on Hippel-Lindau (VHL), multiple endocrine neoplasia type 2 (MEN2), neurofibromatosis type 1 (NF1) and succinate dehydrogenase (SDH) mutation-related tumours. SDH is a mitochondrial complex involved in both Kreb\'s cycle and electron transport chain consisting of different subunits (A-D). Different mutations in various sub-units SDH leads to significant phenotypic heterogeneity, hence has been classified as different paraganglioma syndromes. Herein we review the pathogenesis, inheritance, clinical presentation, diagnosis and management of SDH related paragangliomas.


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