Giant malignant peripheral nerve sheath tumors of the occipital scalp

Background: Malignant peripheral nerve sheath tumors (MPNST) are rare neoplasms, usually arising from peripheral nerves or showing a nerve sheath differentiation. Primary MPNSTs of the scalp is exceptionally rare, and only sporadic cases have been reported recently. Objectives: Report a rare case of giant malignant peripheral nerve sheath tumor (MPNST) beneath occipital scalp, and discuss how to-treat with this kind of tumor. Methods: Descriptive study of a rare case of giant peripheral nerve sheath tumors of occipital scalp without adjuvant treatment with nine months follow up. Results: In a 52-year-old man with MPNSTs beneath occipital scalp, the tumor was treated with complete surgical resection. Histological examination proved that the lesion was a scalp MPNST. The patient was followed up asymptomatic for the following nine months after surgical resection without adjuvant radiotherapy. Conclusion: MPNSTs beneath the occipital scalp should be treated individually, for those well-circumscribed MPNSTs without bone destruction or brain invasion (low-grade tumors), complete surgical resection with clear margins (if possible) is recommended. Otherwise, adjuvant postoperative radiotherapy is necessary.