Neurothekeoma--a benign cutaneous tumor of neural origin

Fifty-three examples of a benign tumor of nerve sheath origin occurring in the dermis are described. Tumors were usually initially noticed by patients in the first or second decade of life. The tumors tended to occur in the central area of the face, arms, and shoulders. There was a 4.3/1 female to male predominance among affected patients. Histologically, these lesions contained nests and cords of large cells in a variably mucinous matrix. In a number of instances, a close histologic relationship existed between tumor cells and small nerves. Despite nuclear atypia and a variable number of mitotic figures, the tumors have behaved in a benign fashion. To our knowledge only two recurred, and inadequate excision was the apparent cause. We propose the name "neurothekeoma" for these tumors (Gr. Oeke, "sheath").