Acromegaly caused by growth hormone-relating hormone in a patient with multiple endocrine neoplasia type I.

A 51-year-old Caucasian man with multiple endocrine neoplasia (MEN) type I syndrome presented with clinical features of acromegaly. Exploration of the pituitary gland only revealed somatotrophic hyperplasia and his plasma growth hormone (GH) levels remained elevated. Production of growth hormone-releasing hormone (GHRH) by an ectopic tumor was suspected and, after additional investigations, a large pancreatic tumor was detected and removed. As the pancreatic tail contained multiple (occult) adenomas, lifelong follow-up was considered necessary. The patient has been recurrence-free for 10 years. All 19 living relatives of this patient were analysed for endocrine disorders related to MEN I syndrome. A brother was found to suffer from peptic ulcer disease caused by hyperparathyroidism and, during screening for other organ involvement associated with the MEN I syndrome, two tumors were found, one (4 cm) in the pancreatic tail region and one in the right adrenal gland. To date, six other family members have been found to suffer or have suffered from hyperparathyroidism and in a male subject, a prolactinoma and hyperparathyroidism were detected.