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Pheochromocytoma is a paraganglioma arising in the adrenal gland. About 10% of cases involve extra-adrenal locations. Some pheochromocytomas are associated with neurofibromatosis type 1, von Hippel-Lindau disease, renal artery dysplasia, adrenal cortical tumors, and other paragangliomas. Familial cases may be seen in the setting of MEN type 2a or type 2b syndromes. Such cases usually occur in a younger age group, are more commonly bilateral, and less likely to be malignant. Grossly, pheochromocytomas are well-circumscribed and encapsulated. They are soft, yellow-tan to slightly brown. Larger tumors may show areas of hemorrhage, cyst formation, and necrosis. A thin compressed rim of bright yellow adrenal cortex is often seen stretched over the tumor. Image courtesy of Dr. Jean-Christophe Fournet, Paris, France; humpath.com; Used with permission

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