Malignant peripheral nerve sheath tumor (
MPNST) is exceedingly rare to occur intracerebrally. The
incidence of this subtype is 0.001%. Their typical origin is from
peripheral nerves of the
limbs, trunk, and lumbar and brachial plexuses. It carries a poor
prognosis despite aggressive management due to high chances of local
recurrence. We present a case of
MPNST occurring intracerebrally in a 60-year-old
male patient. Imaging showed a 63 × 74 × 46 mm mass lesion in the left temporoparietal region with mass effect. The
patient underwent left temporoparietal
craniotomy with maximum gross resection of the
tumor. Histopathology was suggestive of spindle
cell neoplasm. On
immunohistochemistry,
cells were positive for S-100 and SOX-10, which were consistent with the
diagnosis of
MPNST. The post-operative contrast-enhanced
magnetic resonance imaging of
brain showed gross residual
disease. Post-operative
radiotherapy was given of intensity-modulated
radiation therapy followed by
chemotherapy of
doxorubicin and
ifosfamide