Genetic pheochromocytoma/paraganglioma– A review

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