Atypical neurothekeoma: a new case and review of the literature

Background:  Neurothekeoma is a rare neoplasm ascribed into the broad category of benign peripheral nerve sheath tumors. The atypical cellular variant of this neoplasm, showing a peculiar histological pattern, has been very rarely reported in literature. Methods:  The clinical, histopathological and immunohistochemical profile of a new case of atypical cellular neurothekeoma in a 32-year-old woman is reported. Results:  The described neoplasm showed the histological features of atypical variant of cellular neurothekeoma with high-rate mitotic activity and deep penetration into subcutaneous fat. Conclusions:  Because only few cases of atypical neurothekeoma have been reported in literature, clinical and morphological available data on this neoplasm are very few, and its prognosis remains uncertain; anyway, dermatologists should be aware of histological features of this neoplasm for which a complete surgical excision is recommended.