Malignant peripheral nerve sheath tumors (MPNSTs) are rare neoplasms, usually arising from somatic soft tissues or peripheral nerves. The incidence of MPNSTs is approximately 0.001% in the general population. MPNSTs are mainly located in the buttocks, thighs, brachial plexus, and paraspinal region. Scalp is an unusual and surgically challenging site of occurrence. We report a case of giant recurrent MPNST in the left fronto-parietal region, with intracranial extension, in a 58 year old female which was excised completely along with the involved overlying skin, and reconstruction was done for the bony and skin defect. She was given adjuvant radiotherapy. She is doing well after treatment and is in regular follow up. MPNST should be considered by neurosurgeons in the differential of an enlarging scalp soft-tissue lesion with bony and intracranial involvement. Scalp MPNSTs are mostly aggressive lesions, and multimodality approaches are necessary to optimize outcomes
Key words: Malignant peripheral nerve sheath tumors (MPNST), scalp, intracranial, surgery.
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