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
收藏
页码：945 / 947
页数：3
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