Malignant peripheral nerve sheath tumors (MPNSTs) are uncommon, biologically aggressive
soft tissue sarcomas of neural origin that poses tremendous challenges to effective
therapy. MPNSTs are among the most challenging mesenchymal malignancies to treat with
poor prognosis. They usually affect young and middle-aged adults, tend toward early
metastasis, and often demonstrate resistance to chemotherapy. We present a case of
a 23-year-old female who initially presented with the right temporal swelling for
1 month associated with constitutional symptom which progressively worsening. The
right craniotomy and excision biopsy were done with histopathological examination
results suggestive of MPNST. Thorax-abdominal-pelvic computed tomography and magnetic
resonance imaging further revealed multiple metastatic lesions involving spine, retroperitoneal,
pelvic, chest wall, and lungs. This case illustrates the typical presentation of MPNST
with its known poorly outcome.
Key-words:
Malignant peripheral nerve sheath tumors - neurofibromatosis type 1 - prognosis -
survival
