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| Adrenal Pheochromocytoma | ||
| Etiology Mutation in the RET oncogene (a receptor tyrosine kinase) or the MEN2 gene. | ||
| Pathogenesis Unknown Overproduction of unregulated catechols causes striking clinical symptoms, | ||
| Epidemiology 800 cases/year in the U.S. Sporadic accounting for <0.3% of hypertension or Associated with von Hippel-Lindau syndrome or Associated with multiple endocrine neoplasia (MEN) type IIa or May be associated with neurofibromatosis I | ||
| General Gross Description Located in the adrenal medulla surrounded by rim of normal compressed adrenal Vary in size Usually hemorrhagic and necrotic Dichromate fixatives oxidize catechols to brown black pigment Examples: | ||
| General Microscopic Description Cells with frequently pleomorphic nuclei and brown secretory vacuoles Richly vascular stroma Cells in cords or nests Cytologic atypia irrelevant to diagnosis of malignancy Examples: | ||
| References Cotran RS, Kumar V, Robbins SL. Robbins Pathologic Basis of Disease. 5th edition. Philadelphia, W.B. Saunders, 1994, pp. 1162-3 This link will directly take you to the relevant new literature Adrenal Pheochromocytoma
| Synopsis by: Melinda Sanders M.D. (T93000M87000)[152]
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