Multiple Endocrine Neoplasia
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The multiple endocrine neoplasias are familial disorders typically characterized by predisposition to the development of tumors of multiple endocrine organs. These disorders are all inherited in an autosomal dominant fashion, and the tumors tend to be multicentric. The tumors may be benign or malignant and may occur metachronously or synchronously. MEN 1 is characterized by the concurrence of parathyroid hyperplasia, pancreatic islet cell tumors, and pituitary adenomas. MEN 2a consists of medullary carcinoma of the thyroid
, pheochromocytoma, and parathyroid hyperplasia. MEN 2b includes medullary carcinoma of the thyroid, pheochromocytoma, mucosal neuromas, and a distinctive marfanoid habitus. Together, these syndromes encompass much of the spectrum of endocrine neoplasia.
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