title: Adrenal medullary disease in multiple endocrine neoplasia, type 2: pheochromocytoma and its precursors.
abstract: Multiple endocrine neoplasia, type 2 (MEN-type 2), designates the syndrome of medullary thyroid carcinoma, pheochromocytoma, and occasional parathyroid hyperplasia. The thyroid carcinoma, which is usually bilateral and multicentric, is preceded by multifocal C-cell hyperplasia. The adrenal gland manifests pheochromocytoma, which is frequently bilateral and multicentric, and may be malignant. To test the hypothesis that diffuse adrenal medullary hyperplasia is a precursor of pheochromocytoma in this syndrome, we studied the adrenal glands of 19 patients who had MEN-type 2. The findings in the adrenal medulla in these 19 patients were: synchronous bilateral pheochromocytoma in 9 (metastatic in 3); asynchronous bilateral pheochromocytoma in 1 (metastatic); unilateral pheochromocytoma with contralateral diffuse and nodular hyperplasia in 2; unilateral pheochromocytoma with contralateral diffuse hyperplasia in 2; unilateral pheochromocytoma in 1; bilateral nodular hyperplasia in 1; bilateral diffuse hyperplasia in 1; and no abnormality in 2. This spectrum of adrenal medullary pathology suggests that diffuse and nodular medullary hyperplasia are precursors of pheochromocytoma in MEN-type 2.
mesh_terms: D000310:Adrenal Gland Neoplasms / Q000098:blood supply / Q000150:complications* / Q000473:pathology; D000313:Adrenal Medulla / Q000473:pathology; D002467:Cell Nucleus / Q000473:pathology; D003593:Cytoplasm / Q000473:pathology; D005260:Female; D006801:Humans; D006965:Hyperplasia; D008297:Male; D008938:Mitosis; D008954:Models, Biological; D009362:Neoplasm Metastasis; D009378:Neoplasms, Multiple Primary; D010673:Pheochromocytoma / Q000150:complications* / Q000209:etiology / Q000473:pathology; D011230:Precancerous Conditions; D013577:Syndrome; D013964:Thyroid Neoplasms / Q000150:complications*