From WikiProjectMed
Jump to navigation Jump to search
Neurothekeoma histology slide

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.[1] Microscopically, the lesions described closely resembled the tumor, "nerve sheath myxoma", an entity first described by Harkin and Reed.[2] The latter had, through the years, been variously described as "Bizarre cutaneous neurofibroma",[3] "Myxoma of nerve sheath",[3] and "Pacinian neurofibroma".[3]

Clinically, neurothekeomas present as a solitary nodule of the skin.[4] The most common sites of occurrence are the head and neck and the extremities.[4] The lesions range in size from about 0.5 cm. to more than 3 cm.[4] The average patient age is about 25 years, but neurothkeomas may occur at any age.[4] Women are affected about more often; the male to female ratio is approximately 1:2.[4]

Microscopically, neurothekeoma consists of closely aggregated bundles or fascicles of spindle-shaped cells.[5] The fascicles may or may not have a myxoid background.[5]

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.[4] Consequently, neurothekeoma and nerve sheath myxoma are likely not related histogenetically, although they are similar in appearance and in behavior.[4]

Microscopy of nerve sheath myxoma, red arrow indicates the tumoral tissue

See also


  1. Gallager RL, Helwig EB (December 1980). "Neurothekeoma-a benign cutaneous tumor of neural origin". American Journal of Clinical Pathology. 74 (6): 759–764. doi:10.1093/ajcp/74.6.759. PMID 7446487.
  2. Harkin, James C.; Richard J. Reed (1969), Tumors of the Peripheral Nervous System, in Armed Forces Institute of Pathology, ed: Atlas of Tumor Pathology, 2nd series, fascicle 3, Washington, D.C.: Armed Forces Institute of Pathology
  3. 3.0 3.1 3.2 Pulitzer DR, Reed RJ (October 1985). "Nerve-sheath myxoma (perineurial myxoma)". American Journal of Dermatopathology. 7 (5): 407–421. PMID 4091218.
  4. 4.0 4.1 4.2 4.3 4.4 4.5 4.6 Fetsch JF, Laskin WB, Hallman JR, Lupton GP, Miettinen M (July 2007). "Neurothekeoma: an analysis of 178 tumors with detailed immunohistochemical data and long-term patient follow-up information". American Journal of Surgical Pathology. 31 (7): 1103–1114. doi:10.1097/pas.0b013e31802d96af. PMID 17592278. Archived from the original on 2022-02-23. Retrieved 2020-11-27.
  5. 5.0 5.1 Reed, Richard J.; Pulitzer, Donald R. (2008), "Chapter 35: Tumors of Neural Tissue", in Elder D; et al. (eds.), Lever's Histopathology of the Skin (Ninth ed.), Philadelphia: Lippincott Raven, ISBN 978-0781773638