MALIGNANT PERIPHERAL-NERVE SHEATH TUMORS DEVELOPING MULTIFOCALLY IN THE CENTRAL-NERVOUS-SYSTEM IN A PATIENT WITH NEUROFIBROMATOSIS TYPE-2

We describe autopsy findings of multifocal malignant peripheral nerve sheath tumors (MPNSTs) appearing in the central nervous system in a 45 -year-old Japanese female with neurofibromatosis type 2. Multiple MPNS Ts were detected in both III and VIII, left IV, and V cranial nerves, and a number of nerve roots of the spinal cord. Neurofibromata were on the other hand evident on some nerve roots of the spinal cord and fem oral and sciatic nerves. Our results suggest that a mutation of p53 ge ne may have played a role in the malignant transformation of nerve tum ors in this patient since p53 protein was immunohistochemically detect ed in MPNST cells but not in tumor cells of the neurofibromata.