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[第一型多发性内分泌腺瘤病(韦尔默综合征)]

[Multiple endocrine neoplasia of the first type (Wermer's syndrome)].

作者信息

Kuzin N M, Egorov A V, Kuznetsov N S, Lotov A N, Lakreeva M G

出版信息

Klin Med (Mosk). 2000;78(10):28-31.

PMID:11220895
Abstract

11 patients with Wermer's syndrome (WS) aged 24-67 years were treated. They had a total of 30 tumors: 4(13.3%) hypophyseal, 11(36.7%) pancreatic, 9(30%) parathyroid, 4(13.3%) adrenal and 2(6.7%) duodenal. Each patient had two tumors minimum and 8 tumors maximum. In 8 cases WS presented clinically as hypoglycemia, in 3 cases as Zollinger-Ellison syndrome. The diagnosis was made using ultrasonography, CT, MRT and angiography. Cytogenetic examination was made in 2 cases. All the patients were operated. Six of them were operated two times, one--four times. The following surgery was made: 4 removals of parathyroid adenomas, 4 adrenalectomies, 1 removal of the hypophysis, 4 enucleations of pancreatic insulinomas, 5 distal hemipancreatectomies, 1 distal subtotal resection of the pancreas, 1 gastropancreatoduodenal resection. Clinically symptomatic tumors should be operated first.

摘要

对11例年龄在24至67岁之间的韦默综合征(WS)患者进行了治疗。他们共有30个肿瘤:4个(13.3%)垂体瘤、11个(36.7%)胰腺瘤、9个(30%)甲状旁腺瘤、4个(13.3%)肾上腺瘤和2个(6.7%)十二指肠瘤。每位患者最少有两个肿瘤,最多有8个肿瘤。8例WS临床上表现为低血糖症,3例表现为佐林格-埃利森综合征。诊断采用超声检查、CT、磁共振成像(MRT)和血管造影。2例进行了细胞遗传学检查。所有患者均接受了手术。其中6例接受了两次手术,1例接受了四次手术。实施了以下手术:4例切除甲状旁腺腺瘤、4例肾上腺切除术、1例垂体切除术、4例摘除胰腺胰岛素瘤、5例胰腺远端半切除术、1例胰腺远端次全切除术、1例胃胰十二指肠切除术。临床上有症状的肿瘤应首先进行手术。

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