A malignant peripheral nerve sheath tumor of the mediastinum in a patient with neurofibromatosis type 1: Report of a case

被引：4

作者：

Kawachi, Riken ^{[1
]}

Takei, Hidefumi ^{[1
]}

Furuyashiki, Goh ^{[1
]}

Koshi-Ishi, Yoshihiko ^{[1
]}

Goya, Tomoyuki ^{[1
]}

机构：

[1] Kyorin Univ, Sch Med, Gen Thorac Surg Div, Dept Surg, Tokyo 1818611, Japan

来源：

SURGERY TODAY | 2008年 / 38卷 / 10期

关键词：

malignant peripheral nerve sheath tumor; neurofibromatosis type 1; von Recklinghausen's disease; mediastinal tumor;

D O I：

10.1007/s00595-008-3764-0

中图分类号：

R61 [外科手术学];

学科分类号：

摘要：

Malignant peripheral nerve sheath tumor (MPNST) is rare, but its association with neurofibromatosis type 1 (NF1) is well known. A 56-year-old man with NF1 was referred to our hospital for investigation of dyspnea. Computed tomography showed a huge mass occupying the right thorax and invading the inferior vena cava. A salvage resection was performed to alleviate the developing hypoxemia. Histological examination confirmed an MPNST. The tumor regrew rapidly and the patient died 6 weeks after the resection. This case report reinforces the importance of monitoring patients with NF1 carefully because of their increased risk of the development of malignant neoplasms.

引用

页码：945 / 947

页数：3

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