Document Detail

A malignant peripheral nerve sheath tumor of the mediastinum in a patient with neurofibromatosis type 1: report of a case.
MedLine Citation:
PMID:  18820872     Owner:  NLM     Status:  MEDLINE    
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.
Riken Kawachi; Hidefumi Takei; Goh Furuyashiki; Yoshihiko Koshi-Ishi; Tomoyuki Goya
Publication Detail:
Type:  Case Reports; Journal Article     Date:  2008-09-27
Journal Detail:
Title:  Surgery today     Volume:  38     ISSN:  0941-1291     ISO Abbreviation:  Surg. Today     Publication Date:  2008  
Date Detail:
Created Date:  2008-09-29     Completed Date:  2009-03-31     Revised Date:  -    
Medline Journal Info:
Nlm Unique ID:  9204360     Medline TA:  Surg Today     Country:  Japan    
Other Details:
Languages:  eng     Pagination:  945-7     Citation Subset:  IM    
Department of Surgery, Kyorin University School of Medicine, 6-20-2 Shinkawa, Mitaka, Tokyo, 181-8611, Japan.
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MeSH Terms
Fatal Outcome
Mediastinal Neoplasms / complications*,  radiography,  surgery*
Middle Aged
Nerve Sheath Neoplasms / complications*,  radiography,  surgery*
Neurofibromatosis 1 / complications*
Tomography, X-Ray Computed

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