A patient with MEN1 syndrome (menin mutation) is most likely to develop which combination of endocrine tumors?

• A Pheochromocytoma, medullary thyroid carcinoma, and parathyroid hyperplasia
• B Pheochromocytoma, parathyroid adenoma, and vestibular schwannoma
• C Adrenocortical carcinoma, pheochromocytoma, and pituitary carcinoma
• D Parathyroid adenoma/hyperplasia, pituitary adenoma, and pancreatic neuroendocrine tumor ✓

Explanation

Multiple endocrine neoplasia type 1 (MEN1) is caused by loss-of-function mutations in the MEN1 gene encoding menin, a chromatin-associated tumor suppressor. The classic triad ('3 Ps') involves: parathyroid (90%+ of patients — parathyroid adenomas causing hypercalcemia), pituitary (prolactinoma or GH-secreting adenoma, ~30%), and pancreatic/duodenal neuroendocrine tumors (gastrinoma causing Zollinger-Ellison syndrome, insulinoma, VIPoma, ~60%). MEN2A features medullary thyroid carcinoma, pheochromocytoma, and parathyroid hyperplasia (RET mutation); MEN2B features MTC, pheochromocytoma, and marfanoid habitus with mucosal neuromas.

Reference: Robbins & Cotran Pathologic Basis of Disease, 10th ed.

High-yield for: NEET PGINI-CETNExTFMGEUSMLEPLABMRCP

Written and medically reviewed by the StethoPrep medical team.