Multiple Endocrine Neoplasia Syndromes

MULTIPLE ENDOCRINE NEOPLASIA SYNDROMES

Multiple endocrine neoplasia (MEN) syndromes are autosomal dominant conditions with incomplete penetrance that are characterized by hyperplasia and tumors of endocrine glands occurring at a young age.

•         MEN 1 (Werner syndrome) features tumors of the pituitary gland, parathy-roids, and pancreas.

•         Associated with peptic ulcers and Zollinger-Ellison syndrome

Affected gene is MEN1, a tumor suppressor gene which encodes a nuclear protein called menin

•         MEN 2A (Sipple syndrome) features medullary carcinoma of the thyroid, pheo-chromocytoma, and parathyroid hyperplasia or adenoma.

Mutation of RET proto-oncogene

•         MEN 2B features medullary carcinoma of the thyroid, pheochromocytoma, and mucocutaneous neuromas.

•         Mutation of RET proto-oncogene