Malignant Peripheral Nerve Sheath Tumor with Neurofibromatosis Type 1 : A 2-Case Report and Review of the Literature
2001
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.
Key word : Malignant Peripheral Nerve Sheath Tumor, Neurofibromatosis Type
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