von Hippel-Lindau disease is an inherited disorder that affects multiple organs and is characterized by abnormal growth of blood vessels. In addition to pheochromocytoma, patients with von Hippel-Lindau disease have a predisposition to retinal angiomas, central nervous system hemangioblastomas, renal cell carcinomas, islet cell tumors of the pancreas, and cysts of the kidney, pancreas, and epididymis. MEN type II (both IIa and IIb), not MEN type I, is associated with pheochromocytoma. Neurofibromatosis 2 is an autosomal dominant syndrome characterized by multiple schwannomas, meningiomas, and ependymomas. Beckwith-Wiedemann syndrome is a contiguous gene syndrome associated with adrenocortical carcinoma. Patients with hereditary pheochromocytoma are more likely to have multiple bilateral, benign lesions, and these lesions are more likely to be extra-adrenal. MIBG, a compound structurally related to norepinephrine, is taken up by adrenergic tissues and can be used to help localize multiple lesions. MIBG scanning should be strongly considered in patients with suspected hereditary pheochromocytoma.
- von Hippel-Lindau disease.
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