What is "neurothekeoma"

n. A myxoma composed of cords and nests of large cells packed among collagen bundles in close proximity to small nerves.

Neurothekeoma is a benign cutaneous tumor first described by Gallager and Helwig, who proposed the term in order to reflect the presumed origin of the lesion from nerve sheath. Microscopically, the lesions described closely resembled the tumor, “nerve sheath myxoma,” an entity first described by Harkin and Reed. The latter had, through the years, been variously described as “Bizarre cutaneous neurofibroma,” “Myxoma of nerve sheath,” and “Pacinian neurofibroma.”

Clinically, neurothekeomas present as a solitary nodule of the skin The most common sites of occurrence are the head and neck and the extremities. The lesions range in size from about 0.5 cm. to more than 3 cm. The average patient age is about 25 years, but neurothkeomas may occur at any age. Women are affected about more often; the male to female ratio is approximately 1:2.

Microscopically, neurothekeoma consists of closely aggregated bundles or fascicles of spindle-shaped cells. The fascicles may or may not have a myxoid background.

Since the time of their first description, it has been reported that neurothekeomas are likely not of nerve sheath origin, as implied by the term. Consequently, neurothekeoma and nerve sheath myxoma are likely not related histogenetically, although they are similar in appearance and in behavior.