Classic neurothekeoma (nerve sheath myxoma) and cellular neurothekeoma of the oral mucosa: immunohistochemical profiles

J Oral Pathol Med. 2011 Feb;40(2):174-80. doi: 10.1111/j.1600-0714.2010.00952.x. Epub 2010 Oct 4.


Background: Classic neurothekeoma (nerve sheath myxoma) is regarded as being a true benign cutaneous tumor of nerve sheath origin. Cellular neurothekeoma was separated from the classic type by histogenesis, morphology and immunophenotype. Whether cellular neurothekeoma represents a continuum within the spectrum of classic neurothekeoma or is an independent entity is controversial. Only a small number of classic neurothekeomas of the oral mucosa have been reported and there are even fewer publications on cellular neurothekeoma. We analyzed a series of oral neurothekeomas (classic and cellular) with a panel of neural and other mesenchymal markers to enhance their diagnosis and classification.

Methods: One cellular and three classic neurothekeomas were submitted to a panel of immunohistochemical stains with antibodies against S100, S100A6, NSE, NKI/C3, PGP9.5, α-SMA, HHF-35, CD68 and vimentin. Two cases of neurofibroma (plexiform type), representing a true lesion of neural origin, served as control.

Results: The cellular neurothekeoma yielded a positive immunoreaction for S100A6 and NKI/C3 and a negative immunoreaction for S-100. The classic neurothekeomas demonstrated a positive reaction for S-100 and S100A6, but a negative one for NKI/C3. Other markers were non-contributory to distinguishing between these types of lesions.

Conclusions: The small number of reported oral neurothekeomas (classic and cellular) could be due, in part, to the lack of recognition of their particular morphologic and immunohistochemical features. Our results indicate that testing for NKI/C3 immunoreactivity may be of value in distinguishing between cellular and classic neurothekeoma.

Publication types

  • Research Support, Non-U.S. Gov't

MeSH terms

  • Adult
  • Biomarkers, Tumor / analysis
  • Cell Cycle Proteins / analysis
  • Child
  • Female
  • Humans
  • Immunohistochemistry
  • Male
  • Mouth Mucosa / pathology
  • Mouth Neoplasms / chemistry*
  • Mouth Neoplasms / classification
  • Mouth Neoplasms / pathology*
  • NK Cell Lectin-Like Receptor Subfamily B / analysis
  • Neurothekeoma / chemistry*
  • Neurothekeoma / classification
  • Neurothekeoma / pathology*
  • S100 Calcium Binding Protein A6
  • S100 Proteins / analysis
  • Young Adult


  • Biomarkers, Tumor
  • Cell Cycle Proteins
  • NK Cell Lectin-Like Receptor Subfamily B
  • S100 Calcium Binding Protein A6
  • S100 Proteins
  • S100A6 protein, human