Primary intracranial malignant peripheral nerve sheath tumour responding to chemotherapy

Malignant peripheral nerve sheath tumours (MPNST) are a rare variety of soft tissue sarcomas (STS) arising from major peripheral nerve branches and typically located in the lower extremity, chest wall or the retroperitoneum. It is a biologically aggressive neoplasm for which the treatment of choice is surgery, but usually requires a multimodality approach, having been generally labelled as chemoresistant. We present a case of MPNST located intracranially with a good response to chemotherapy.