[1] First described by Richard L Gallager and Elson B. Helwig, who proposed the term in order to reflect the presumed origin of the lesion from nerve sheath.

[2] Microscopically, the lesions described closely resembled the tumor, "nerve sheath myxoma (NSM)", an entity first described by Harkin and Reed.

[12][13] In 2011, Sheth et al. used microarray analysis to distinguish between neurothekeoma and nerve sheath myxoma based on the genetic expression of the cells.

[17] Lesions are often a singular subcutaneous dome-shaped papule or nodule measuring less than 2 cm and may be skin-colored, pink to tan, or red to brown.

The lesions tend to grow slowly and are usually superficially located, with rare, deeper involvement of subcutaneous fat or skeletal muscle.

[23] There have been reports of neurothekeomas exhibiting atypical features, raising concern for their potential for aggression even though the majority of these tumors are small (less than 1 cm), have relatively bland histology, little to no cytologic atypia, and only minimal extension into surrounding fat or skeletal muscle.

Clinical size more than 1 cm, pleomorphism and enhanced mitotic activity in the cytology, infiltration into skeletal muscle or subcutaneous fat, and vascular invasion are reported as unusual characteristics.