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 MPNSTs 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 femoral and sciatic nerves. Our results suggest that a mutation of p53 gene may have played a role in the malignant transformation of nerve tumors in this patient since p53 protein was immunohistochemically detected in MPNST cells but not in tumor cells of the neurofibromata.
Higami
Y
Y
Department of Pathology, Nagasaki University School of Medicine, Japan.
Shimokawa
I
I
Kishikawa
M
M
Okimoto
T
T
Ohtani
H
H
Tomita
M
M
Tsujino
A
A
Ikeda
T
T
D017809:Fatal Outcome; D005260:Female; D006801:Humans; D007150:Immunohistochemistry; D008279:Magnetic Resonance Imaging; D008875:Middle Aged; D009378:Neoplasms, Multiple Primary / Q000737:chemistry / Q000150:complications / Q000175:diagnosis* / Q000473:pathology; D018317:Nerve Sheath Neoplasms / Q000737:chemistry / Q000150:complications / Q000175:diagnosis* / Q000473:pathology; D016518:Neurofibromatosis 2 / Q000150:complications / Q000473:pathology*; D010524:Peripheral Nervous System Neoplasms / Q000737:chemistry / Q000150:complications / Q000175:diagnosis* / Q000473:pathology; D009418:S100 Proteins / Q000032:analysis; D016159:Tumor Suppressor Protein p53 / Q000032:analysis
D009418:S100 Proteins; D016159:Tumor Suppressor Protein p53