Neurofibrosarcoma of skin and subcutaneous tissues.

In this report, we describe 13 cases of primary neurofibrosarcoma of the skin. The tumor presumably arises from small cutaneous nerves, is locally aggressive, and has a potential for metastasis. Characteristic histopathologic features include proliferating atypical spindle cells with slender wavy and pointed nuclei; hypocellular areas with loose, myxoid stroma; and areas of organoid organization such as palisading, whorly, storiform, and tactile body-like formations. The S-100 stain is positive in about 60% of cases. In the current series, most tumors arose in deep dermis and were grade 2 malignant lesions with a moderate degree of cytologic atypia and 2 or fewer mitoses in 10 high-power fields. Three patients died of their malignant lesion. Only two tumors metastasized. Of the 10 patients who had local recurrence, 5 had multiple recurrent lesions. Neurofibrosarcoma should be considered in the differential diagnosis of malignant tumors of the skin. A complete surgical resection of the primary tumor with adequate margins of surrounding normal-appearing tissue is advised.