1型多发性内分泌腺瘤病患者的巨大胰岛素瘤：病例报告

Giant insulinoma in a patient with multiple endocrine neoplasia-type I: a case report.

作者信息

Kataoka H, Otsuka F, Yamauchi T, Kishida M, Takahashi M, Tamiya T, Mimura Y, Ogura T, Makino H

机构信息

Department of Medicine III, Okayama University Medical School, Japan.

出版信息

Endocr J. 1999 Jun;46(3):429-35. doi: 10.1507/endocrj.46.429.

DOI:10.1507/endocrj.46.429

PMID:10503996

Abstract

We report a case of giant cystic insulinoma constituting part of multiple endocrine neoplasia (MEN) type I. A 29-year-old Japanese man presented with a history of recurrent hypoglycemic attacks. Endocrine examination showed hyperinsulinemia discordant with hypoglycemia, and a giant cystic insulinoma (11 x 10 cm) located in the pancreatic tail was detected radiologically. Hyperprolactinemia due to pituitary adenoma and hyperparathyroidism due to parathyroid hyperplasia were also present. The insulinoma, prolactinoma and hyperplastic parathyroid gland were surgically removed. Fluorescent microsatellite analysis detected loss of heterozygosity (LOH) in chromosome 11q13 in DNA samples from all resected tissues but not from white blood cells. This is a rare case of MEN type I because of the giant cystic insulinoma and the evidence of common LOH detected in all MEN type I tissues.

摘要

我们报告一例构成多发性内分泌腺瘤病（MEN）I型一部分的巨大囊性胰岛素瘤病例。一名29岁的日本男性有反复低血糖发作史。内分泌检查显示高胰岛素血症与低血糖不一致，影像学检查发现胰腺尾部有一个巨大囊性胰岛素瘤（11×10 cm）。还存在垂体腺瘤导致的高催乳素血症和甲状旁腺增生导致的甲状旁腺功能亢进。胰岛素瘤、催乳素瘤和增生的甲状旁腺均通过手术切除。荧光微卫星分析在所有切除组织的DNA样本中检测到11q13染色体杂合性缺失（LOH），但在白细胞中未检测到。这是一例罕见的MEN I型病例，原因是存在巨大囊性胰岛素瘤以及在所有MEN I型组织中检测到常见的LOH证据。