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Asian J Neurosurg. 2019 Jan-Mar;14(1):283-285. doi: 10.4103/ajns.AJNS_332_17.

Malignant Peripheral Nerve Sheath Tumor: Treat or Not Treat?

Author information

1
Department of Neurosurgery, Hospital Raja Permaisuri Bainun, Ipoh, Perak, Malaysia.

Abstract

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.

KEYWORDS:

Malignant peripheral nerve sheath tumors; neurofibromatosis type 1; prognosis; survival

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