RESUMO
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.
RESUMO
Diffuse neurofibroma is an uncommon variant of neurofibroma that usually presents on the head and neck. It is common in children and young adults. Clinically, the lesion presents as large, poorly circumscribed swelling. A 25 year-old female presented with diffuse swelling on the occipital region of the scalp for several years. Magnetic resonance imaging showed 8x9 cm sized, ill defined infiltration on the occipital scalp. The histologic examination revealed unencapsulated loosely arranged spindle cells within the dermis and subcutaneous tissue. Meissnerian differentiation was observed within the tumor cells. The tumor cells were positive for S-100 protein. We report a case of diffuse neurofibroma on the occipital region of the scalp.