MEN

A group of autosomal-dominant syndromes characterized by multiple endocrine tumors due to defective tumor suppressor genes.

MEN 1 (Wermer's): tumors of parathyroid (95%), pancreas (30-80%), pituitary. Carcinoid, adrenal adenomas, subcutaneous lipomas.

• • Screen if there is a positive family history. Check Sr. Ca, PTH, gastrin, and prolactin.

  • MENIN gene

MEN 2A (Sipple's): hyperparathyroidism (25%), medullary thyroid cancers (80-90%), pheochromocytomas (40%).

• • RET protoncogene

  • Screen with RET proto-oncogene. If mutation is found obtain basal stimulated calcitonin, plasma or urine metanephrines, Sr. Ca, PTH, gastrin.

MEN 2B: medullary thyroid cancers (100%), pheochromocytomas (50%), Marfanoid habitus (75%), mucosal neuromas (100%), intestinal ganglioneuroma, delayed puberty.

• • RET protoconcogene

Pancreatic tumors: gastrinomas, associated with ZES, insulinomas, glucagonomas, VIPomas, on non-functioning tumors.