제1형 다발성 내분비 선종의 임상양상

Clinical Manifestation of Multiple Endocrine Neoplasia Type I

Purpose: Multiple endocrine neoplasia type I is rarely reported in Korea. The purpose of this study is to analyze the clinical features and the treatment of MEN1 which had been carried out in our institution. Methods: Eight patients underwent surgery for MEN1-related hyperparathyroidism, duodenopancreatic tumors and pituitary tumors at our institution and were reviewed retrospectively. Results: Of the eight patients, all patients had hyperparathyroidism. There were 5 total parathyroidectomy patients and 3 subtotal parathyroidectomy patients, none of them had persistent and recurrent hyperparathyroidism after operation. However, one of total parathyroidectomy patients had persistent hypoparathyroidism. Of the 8 patients, seven had duodenopancreatic tumors; four of them had pancreatic islet-cell tumors, two had malignant gastrinoma and one had Zollinger- Ellision syndrome. Pituitary tumors were observed in 6 patients among the 8; four of them had prolactinoma, and 2 had adenoma which was confirmed by magnetic resonance imaging. Of the eight patient, only one patient was confirmed to have another MEN1 family member and the mutation of MEN1 gene on chromosome 11q13 was proved on gene study.Conclusion: The clinical features of these patients were similar to those in other reports and the results of surgical treatment were relatively satisfactory. However, the detection of other MEN1 patients among the family members was relatively poor. Close follow up of family members and gene study will be required. (Korean J Endocrine Surg 2004;4: 31-35)