Pheochromocytoma

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1. 10% of patients with vonHippel Lindau (VHL) have pheochromocytoma while 23% of patients diagnosed initially as having pheochromocytoma will have either the VHL complex or multiple endocrine neoplasia (MEN) IIa
2. 10% of pheochromocytomas are malignant
3. symptoms due to catecholamine (epinephrine and norepinepherine) secretion and include: episodic headaches, excessive sweating, palpitation, nervousness, and tremor; headaches in a patient with VHL may be due to cerebellar tumor with increased intracranial pressure or to catecholamine excess from the pheochromocytoma; blood pressure may rise greatly during procedure for pheochromocytoma
4. best screening test: urinary metanephrine assay; may also screen with vanillylmandelic acid (VMA) and catecholamines
5. treatment: resection with a 90% cure rate