Pheochromocytoma is associated with:
High-Yield Explanation
About 25-33% of patients with a Pheochromocytoma or paraganglioma have an inherited syndrome. Neurofibromatosis type 1 (NF 1) was the first described pheochromocytoma-associated syndrome. The NF1 gene functions as a tumor suppressor by regulating the Ras signaling cascade. Classic features of neurofibromatosis include multiple neurofibromas, cafe au lait spots, axillary freckling of the skin, and Lisch nodules of the iris. Pheochromocytomas occur in only about 1% of these patients and are located predominantly in the adrenals.