Neurothekeoma—A Benign Cutaneous Tumor of Neural Origin

TLDR

The authors propose the name “neurothekeoma” for these benign nerve‑sheath tumors. They describe fifty‑three cases of a dermal tumor of nerve‑sheath origin. The tumors were usually first noticed in the first or second decade of life, predominantly in females (4.3:1), located mainly on the central face, arms, and shoulders, and histologically comprised nests and cords of large cells in a mucinous matrix with occasional close association to small nerves; despite nuclear atypia and variable mitoses, they behaved benignly with only two recurrences linked to incomplete excision.

Abstract

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. ϴeke, 'sheath').