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[一例伴有多种腹部疾病及肾细胞癌的冯·希佩尔-林道病]

[A case of von Hippel-Lindau disease with multiple abdominal disorders and renal cell carcinoma].

作者信息

Funahashi H, Tuga T, Oike E, Tuji S, Tubouti N, Nagura H, Nakanishi H

机构信息

Dept. of Surgery, Nakatsugawa City Hospital.

出版信息

Gan No Rinsho. 1987 Oct;33(12):1488-93.

PMID:2824879
Abstract

A 52-year-old male underwent right nephrectomy after a clinical diagnosis of renal cell carcinoma. He suddenly lost consciousness 3 days after the nephrectomy and expired 3 weeks later. An autopsy revealed a hemangio-blastoma in the forehead skin. Multiple cysts, cyst-adenomas and an islet cell tumor were also identified in the pancreas. Considering the presence of blindness and the familiar, prevalence of this disease, his case was diagnosed as a rare manifestation of von Hippel Lindau disease associated with renal cell carcinoma, complicated with abdominal disorders.

摘要

一名52岁男性在临床诊断为肾细胞癌后接受了右肾切除术。肾切除术后3天他突然失去意识,并于3周后死亡。尸检发现前额皮肤有血管母细胞瘤。胰腺中还发现了多个囊肿、囊腺瘤和胰岛细胞瘤。考虑到存在失明以及该疾病的家族患病率,他的病例被诊断为与肾细胞癌相关的von Hippel Lindau病的罕见表现,并伴有腹部疾病。

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