Gastrointestinal manifestations of multiple endocrine neoplasia, type IIB

Abstract

Multiple endocrine neoplasia, type IIB (MEN 2b), is comprised of medullary carcinoma of the thyroid, pheochromocytoma, diffuse ganglioneuromatosis of the gastrointestinal tract, and skeletal abnormalities. Alimentary tract manifestations, especially enlargement of the lips and tongue by proliferative neural tissue, are often the earliest clues to the presence of the syndrome. A patient is presented here who illustrates the chronicity and diversity of symptoms characteristic of MEN 2b. It is important to recognize the typical oral manifestations of this syndrome so that the thyroid gland can be evaluated before the development of disseminated medullary carcinoma.