[A case of malignant peripheral nerve sheath tumor requiring thoracotomy]

A 32-year-old woman with Von Recklinghausen's disease had a malignant peripheral nerve sheath tumor that had progressed to malignancy from a neurofibroma arising in the left phrenic nerve. The tumor was removed combining a neck incision and thoracotomy. It should be more widely recognized that neurofibroma associated with Von Recklinghausen's disease may progress to malignancy. Primary treatment requires a broad surgical excision to obtain an adequate margin.