A Case of Multiple Endocrine Neoplasia Type I Presented with Secondary Amenorrhea and Osteoporosis / 대한내분비학회지

ABSTRACT

MEN type 1 is characterized primarily by the presence of functioning and nonfunctioning tumors or hyperplasia of the pituitary gland, parathyroid glands, and pancreatic islet cells. Pancreatic islet tumors in MEN type 1 produce different kinds of hormone which were pancreatic polypeptide, gastrin, glucagon, insulin and so on. To date, ten cases had been reported in Korea. We report another case with MEN type 1 having prolatin-secreating pituitary adenoma, parathyroid hyperplasia and insulinoma. A 36-year-old woman was admitted because of long-standing amenorrhea and recently diagnosed osteoporosis. Otherwise, she had been in good health except experiencing one episode of loss of consciousness after skipped meal. The blood chemistries were normal except hypercalcemia and hypophosphatemia. Hormonal studies revealed elevated levels of intact PTH and prolactin and decreased value of estradiol with low LH and FSH. The neck CT revealed 1 cm-sized nodule at posterior portion of right thyroid gland and 99mTc-sestamibi sintigraphy showed a increased uptake in left lower and right lower parathyroid glands. The sella MRI showed 0.7 cm-sized enhanced lesion in the left pituitary gland. The ratio of immunoreactive insulin to glucose was elevated and 3-4 pancreatic masses of variable size were identified by endoscopic ultrasonography and angiography. Subtotal parathyroidectomy and pyrolus-preserving pancreaticoduodenectomy with spleen-preserving distal pancreatectomy was done. Postoperative she had been doing well with normocalcemia and normoglycemia. Transsphenoidal adenonectomy was done 5 months later. Histologic examination of removed tissues revealed a single insulinoma, prathyroid hyperplasia and prolactin-secreating pituitary adenoma.