Pheochromocytoma is associated with
Correct Answer: Von Hippel Lindau syndrome
Description: (A) Von Hippel Lindau syndrome # Pheochromocytoma is a rare catecholamine-secreting tumor derived from chromaffin cells. Tumors that arise outside the adrenal gland are termed extra-adrenal pheochromocytomas or paragangliomas. Because of excessive catecholamine secretion, pheochromocytomas may precipitate life-threatening hypertension or cardiac arrhythmias. If the diagnosis of a pheochromocytoma is overlooked, the consequences could be disastrous, even fatal; however, if a pheochromocytoma is found, it is potentially curable.> Pheochromocytomas are rare, reportedly occurring in 0.05-0.2% of hypertensive individuals. Patients may be completely asymptomatic. A retrospective study from the Mayo Clinic revealed that in 50% of cases, the diagnosis was made at autopsy. Approximately 10% of pheochromocytomas are discovered incidentally. Pheochromocytomas may occur in certain familial syndromes, including multiple endocrine neoplasia (MEN) 2A and 2B, neurofibromatosis, and von Hippel-Lindau (VHL) disease.> VHL syndrome is associated with pheochromocytomas, cerebellar hemangioblastomas, and renal cell carcinoma
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