Genetic Pheochromocytoma -a Review
Keywords:
Pheochromocytoma, paraganglioma ,germ line mutations ,familial syndromes.Abstract
The prevalence of pheochromcytoma in hypertensive patients is less than 1%.Most PHEOs occur sporadically, but a substantial proportionmay be associated with germ line mutations of genes predisposing to thedevelopment of familial syndromes like multiple endocrineneoplasia(MEN),Von-Hippel Lindau(VHL) disease,neurofibromatosis type 1(NF-1),familial paraganglioma/pheochromocytoma (PGL/PHEO) related to geneticmutation encoding the mitochondrial protein succinate dehdrogenase sub units(SDH-BCD) .Screening for genetic mutation is imperative as it may add more onmanagement and surveillance of this patients .This review summarize therelevant data related to this fascinating clinical entity.
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