A 32-year-old man is evaluated for recurrent peptic ulcer disease and is found to have hypercalcaemia with an elevated PTH. Family history reveals a brother who died of a malignant insulin-secreting tumour. Screening for multiple endocrine neoplasia type 1 is planned. Which gland is most commonly the FIRST to manifest disease in this syndrome?

• A Adrenal cortex, through bilateral macronodular hyperplasia
• B Anterior pituitary, through a prolactin-secreting adenoma
• C Pancreas, through a gastrin-producing neuroendocrine tumour
• D Parathyroid glands, through four-gland hyperplasia ✓

Explanation

Primary hyperparathyroidism from four-gland hyperplasia occurs in about 90% of MEN1 carriers and is almost always the first and presenting lesion, typically in the third decade. Pancreatic neuroendocrine tumours, chiefly gastrinomas, follow later, and pituitary adenomas are less frequent. Adrenal disease in MEN1 is usually nonfunctional and is not a recognised presenting feature.

Reference: Williams Textbook of Endocrinology, 14th ed.

High-yield for: NEET PGINI-CETNExTFMGEUSMLEPLABMRCP

Written and medically reviewed by the StethoPrep medical team.