Neurothekeoma

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Neurothekeoma is a benign peripheral nerve sheath tumour.

It is also known as nerve sheath myxoma[1] and myxoma of the nerve sheath.

There is growing evidence that neurothekomas and dermal nerve sheath myxomas are distinct entities.[2]

General

  • Rare.
  • Female > male.

Microscopic

Features:[3]

  • Superficial dermal lesion:
    • Usu. lobulated or micronodular architecture - key feature.
      • +/-Focal sheeting.
    • Spindle/epithelioid morphology with pale eosinophilic cytoplasm - key feature.
    • +/-Inflammation around lesion.
    • +/-Surrounded by collagen.

Notes:

  • No atypia.
  • Mitoses rare/none.
  • Often poorly circumscribed.

Subtypes:[4]

  • Cellular.
  • Myxoid.
  • Intermediate.

DDx:

Images

www

IHC

Features:[3]

  • NKI/C3 (AKA NKI-C3) +ve.
  • NSE +/-ve.

Others:[6]

  • Vimentin +ve.
  • CD10 +ve.
  • Microphthalmia transcription factor +ve.
  • PGP9.5 +ve.
  • S100 +ve.[7]
    • -ve in older literature.

See also

References

  1. Laskin, WB.; Fetsch, JF.; Miettinen, M. (Oct 2000). "The "neurothekeoma": immunohistochemical analysis distinguishes the true nerve sheath myxoma from its mimics.". Hum Pathol 31 (10): 1230-41. doi:10.1053/hupa.2000.18474. PMID 11070116.
  2. Sheth, S.; Li, X.; Binder, S.; Dry, SM. (Mar 2011). "Differential gene expression profiles of neurothekeomas and nerve sheath myxomas by microarray analysis.". Mod Pathol 24 (3): 343-54. doi:10.1038/modpathol.2010.203. PMID 21297585.
  3. 3.0 3.1 Hornick JL, Fletcher CD (March 2007). "Cellular neurothekeoma: detailed characterization in a series of 133 cases". Am. J. Surg. Pathol. 31 (3): 329–40. doi:10.1097/01.pas.0000213360.03133.89. PMID 17325474.
  4. Wang AR, May D, Bourne P, Scott G (November 1999). "PGP9.5: a marker for cellular neurothekeoma". Am. J. Surg. Pathol. 23 (11): 1401–7. PMID 10555009.
  5. URL: http://surgpathcriteria.stanford.edu/softfib/angiomatoid_fibrous_histiocytoma/. Accessed on: 11 May 2011.
  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". Am. J. Surg. Pathol. 31 (7): 1103–14. doi:10.1097/PAS.0b013e31802d96af. PMID 17592278.
  7. Busam, Klaus J. (2009). Dermatopathology: A Volume in the Foundations in Diagnostic Pathology Series (1st ed.). Saunders. pp. 541. ISBN 978-0443066542.