Malignant Peripheral Nerve Sheath Tumor with Neurofibromatosis Type 1: A 2-Case Report and Review of the Literature

APICHAT ASAVAMONGKOLKUL,M.D.*, THANJIRA JIRANANTAKAN**, SARANATRA WAIKAKUL, M.D.*, KULLANUCH PHOMPITAKSA, M.D.***, SORRANART MUANGSOMBOON, M.D.****

Affiliation : * Department of Orthopaedic Surgery, ** 61h Year Medical Student, ***Department of Radiology, ****Department of Pathology, Faculty of Medicine Siriraj Hospital, Mahidol University, Bangkok 10700, Thailand. t This paper was presented at the 40th Annual Meeting of Siriraj Scientific Congress, Bangkok, Thailand, March, 2000

AbstractMalignant 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.

Keywords : Malignant Peripheral Nerve Sheath Tumor, Neurofibromatosis Type

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