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肠系膜神经纤维瘤伴冯雷克林霍增氏病:一例报告

Mesenteric neurofibroma with von Recklinghausen's disease: a case report.

作者信息

Yano K, Okamura T, Yoshida Y, Osaki T, Ichiyoshi Y, Yasumoto K

机构信息

Second Department of Surgery, School of Medicine, University of Occupational and Environmental Health, Kitakyushu, Japan.

出版信息

Hepatogastroenterology. 1998 Mar-Apr;45(20):456-8.

PMID:9638426
Abstract

Mesenteric neurofibroma associated with von Recklinghausen's disease is rare. Herein, we present one such case. A 15-year-old mentally retarded Japanese boy presented with destruction of the right 2nd and 3rd ribs on a routine chest roentgenogram. Physical examination revealed a funnel chest and multiple cafe-au-lait spots, but no cutaneous nodules. Although the patient had no symptoms, a computed tomography (CT) and angiogram were performed. There were no definitive findings of malignancy in the tumors. However, since there were two risk factors for malignancy, specifically, a young age at the time of diagnosis and multiple tumors, and coupled with the size of the abdominal tumor which was large, the abdominal mesenteric tumor was removed. Pathological examination showed a neurofibroma with no evidence of malignancy.

摘要

与冯雷克林霍增氏病相关的肠系膜神经纤维瘤很罕见。在此,我们报告这样一例病例。一名15岁智力发育迟缓的日本男孩在常规胸部X线检查中发现右侧第2和第3肋骨破坏。体格检查发现有漏斗胸和多个咖啡牛奶斑,但无皮肤结节。尽管患者没有症状,但还是进行了计算机断层扫描(CT)和血管造影。肿瘤中未发现明确的恶性征象。然而,由于存在两个恶性风险因素,即诊断时年龄小和肿瘤多发,再加上腹部肿瘤体积较大,于是切除了腹部肠系膜肿瘤。病理检查显示为神经纤维瘤,无恶性证据。

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