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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 |
| 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 |
| Clinical Correlation • Familial cases present much earlier than sporadic cases who are usually > 40. • Episodic or paroxysmal hypertension often associated with sweating • Rarely malignant • Malignancy determined by the presence of metastatic disease. |
| References • Cotran RS, Kumar V, Robbins SL. Robbins Pathologic Basis of Disease. 5th edition. Philadelphia, W.B. Saunders, 1994, pp. 1162-3 |
| Adrenal Pheochromocytoma |
| Synopsis by: Melinda Sanders M.D. (T93000M87000)[152] |
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