1型多发性内分泌腺瘤病伴十二指肠类癌肿瘤和高胃泌素血症：1例家族性病例

Multiple endocrine neoplasia type 1 accompanied by duodenal carcinoid tumors and hypergastrinemia: a familial case.

作者信息

Sugimoto K, Oosawa S, Furuta T, Arai H, Sato Y, Ikuma M, Yamada M, Suzuki M, Hanai H, Kaneko E

机构信息

First Department of Medicine, Hamamatsu University, School of Medicine.

出版信息

Intern Med. 1995 Jul;34(7):649-53. doi: 10.2169/internalmedicine.34.649.

DOI:10.2169/internalmedicine.34.649

PMID:7496077

Abstract

A 38-year-old female was admitted for investigation of the cause of hypergastrinemia, hypercalcemia and an elevated plasma parathyroid hormone (PTH) level. Her siblings, elder brother and sister who had duodenal carcinoid tumor with hypergastrinemia and parathyroid adenomas, were diagnosed as multiple endocrine neoplasia (MEN) type 1. In the present patient, endoscopic examination showed a carcinoid tumor of the duodenum, but examinations by computed tomography (CT) and ultrasonography of the abdomen failed to reveal pancreatic lesions. Serum gastrin levels of this patient and her siblings improved to the normal level after resection of carcinoid tumors. The hypergastrinemia accompanying MEN type 1 in these cases might be due to carcinoid tumor of the duodenum.

摘要

一名38岁女性因高胃泌素血症、高钙血症及血浆甲状旁腺激素（PTH）水平升高入院接受病因调查。她的兄弟姐妹，即患有十二指肠类癌瘤伴高胃泌素血症和甲状旁腺腺瘤的哥哥和姐姐，被诊断为1型多发性内分泌肿瘤（MEN）。在本例患者中，内镜检查显示十二指肠类癌瘤，但腹部计算机断层扫描（CT）和超声检查未发现胰腺病变。该患者及其兄弟姐妹在类癌瘤切除后血清胃泌素水平恢复正常。这些病例中伴随1型MEN的高胃泌素血症可能是由于十二指肠类癌瘤所致。