A malignant peripheral nerve sheath tumor of the mediastinum in a patient with neurofibromatosis type 1: report of a case.
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.
Kawachi
Riken
R
Department of Surgery, Kyorin University School of Medicine, 6-20-2 Shinkawa, Mitaka, Tokyo, 181-8611, Japan.
Takei
Hidefumi
H
Furuyashiki
Goh
G
Koshi-Ishi
Yoshihiko
Y
Goya
Tomoyuki
T
D017809:Fatal Outcome; D006801:Humans; D008297:Male; D008479:Mediastinal Neoplasms / Q000150:complications* / Q000000981:diagnostic imaging / Q000601:surgery*; D008875:Middle Aged; D018317:Nerve Sheath Neoplasms / Q000150:complications* / Q000000981:diagnostic imaging / Q000601:surgery*; D009456:Neurofibromatosis 1 / Q000150:complications*; D014057:Tomography, X-Ray Computed