Indian Journal of Endocrine Surgery and Research

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


Succinate Dehydrogenase Mutation and Paraganglioma Syndromes: A Review Article

Pooja Ramakant

Keywords : Paraganglioma, Pheochromocytoma, Succinate dehydrogenase, Endocrine surgery

Citation Information : Ramakant P. 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; The Author(s).


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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