<prompt> Pheochromocytoma is associated withVon Hippel Lindau syndromeMEN-IMultiple neurofibromatosisInsulinomas  <response>: 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 