Giant scalp malignant peripheral nerve sheath tumor with bone involvement: Case report and review of the literature

Background: Malignant peripheral nerve sheath tumors (MPNSTs) constitute a group of malignant neoplasms with neuroectodermal origin. MPNSTs are rare, with an expected incidence of 0.1/100,000 per year. Occurrences of MPNSTs on the scalp are exceptionally rare, with only 26 cases involving the scalp reported in the published literature. We wish to report a rare case of giant scalp MPNST and discuss the management options of this rare entity, along with the literature review pertaining to the same. Case Description: A 50-year-old male presented with a firm to hard swelling on the top of his head for the past 8 months. Magnetic resonance imaging showed an extra-axial, transcalvarial heterogeneously enhancing lesion of size 10 cm × 9 cm × 7 cm in the midline in the frontoparietal convexity. The patient underwent bi-frontal craniectomy with gross total resection of the tumor mass with titanium mesh cranioplasty. His postoperative period was uneventful, and he was discharged on the 7 th postoperative day. Conclusion: The cases of MPNST should be treated by resection of the affected bone and cranioplasty followed by radiation therapy. Chemotherapy is reserved for high-grade lesions with distant metastasis. The overall survival rate for these highly aggressive tumors is frequently restricted, with a reported figure of approximately 30% over 5 years.