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 7th 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.