title: Malignant peripheral nerve sheath tumor with neurofibromatosis type 1: a 2-case report and review of the literature.
abstract: Malignant peripheral nerve sheath tumor is a very rare soft tissue tumor in the general population but there is an increased incidence in patients with neurofibromatosis type 1. Two cases of malignant peripheral nerve sheath tumor associated with neurofibromatosis type 1 whom we were able to follow-up long term are presented. Although wide excision was performed successfully in these patients, they suffered from local recurrence of the tumors shortly after surgery and died with distant metastases. The literature concerning the natural history and the management of this specific condition was reviewed.
authors.affiliation: Department of Orthopaedic Surgery, Faculty of Medicine Siriraj Hospital, Mahidol University, Bangkok, Thailand.
authors.forename: A
authors.initials: A
authors.lastname: Asavamongkolkul
authors.forename: T
authors.initials: T
authors.lastname: Jiranantakan
authors.forename: S
authors.initials: S
authors.lastname: Waikakul
authors.forename: K
authors.initials: K
authors.lastname: Phompitaksa
authors.forename: S
authors.initials: S
authors.lastname: Muangsomboon
mesh_terms: D000328:Adult; D017809:Fatal Outcome; D005260:Female; D006801:Humans; D009364:Neoplasm Recurrence, Local; D009456:Neurofibromatosis 1 / Q000150:complications*; D010524:Peripheral Nervous System Neoplasms / Q000473:pathology / Q000601:surgery