Section: USMLE

37) A patient consults a physician because of a small lesion on the lips that, on biopsy, proves to be a mucosal neuroma. The patient’s mother had medullary carcinoma of the thyroid. In addition to medullary carcinoma of the thyroid, to which of the following conditions would this patient be particularly vulnerable?

1. Gastrinoma
2. Insulinoma
3. Parathyroid adenoma
4. Pheochromocytoma
5. Pituitary adenoma

You should recognize this as a probable case of multiple endocrine neoplasia, specifically, MEN III (formerly MEN II b). Features of this autosomal dominant condition include medullary carcinoma of the thyroid, pheochromocytoma, and oral and intestinal ganglioneuromatosis (including mucosal neuromas).

Gastrinomas, insulinomas, and pituitary adenomas are found in MEN I.

Parathyroid adenomas are found in MEN I and II.