Compendium of Cancer Genome Aberrations

Dermal nerve sheath myxoma

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Soft Tissue and Bone Tumours (Who Classification, 5th ed.)

Primary Author(s)*

Kathleen Schieffer, PhD, FACMG

WHO Classification of Disease

Structure Disease
Book Soft Tissue and Bone Tumours (5th ed.)
Category Soft tissue tumours
Family Peripheral nerve sheath tumours
Type Dermal nerve sheath myxoma
Subtype(s) N/A

Related Terminology

Acceptable N/A
Not Recommended N/A

Other: Previous terminology included “classic or myxoid variant of neurothekeoma”.[1] However, pathological data[2][3][4][5] and molecular data[6] have demonstrated the Schwann cell derivation of dermal nerve sheath myxoma, which is clinically and biologically distinct from neurothekeoma.

Gene Rearrangements

None

Driver Gene Fusion(s) and Common Partner Genes Molecular Pathogenesis Typical Chromosomal Alteration(s) Prevalence -Common >20%, Recurrent 5-20% or Rare <5% (Disease) Diagnostic, Prognostic, and Therapeutic Significance - D, P, T Established Clinical Significance Per Guidelines - Yes or No (Source) Clinical Relevance Details/Other Notes
N/A N/A N/A N/A N/A N/A N/A N/A

Individual Region Genomic Gain/Loss/LOH

None

Chr # Gain, Loss, Amp, LOH Minimal Region Cytoband and/or Genomic Coordinates [Genome Build; Size] Relevant Gene(s) Diagnostic, Prognostic, and Therapeutic Significance - D, P, T Established Clinical Significance Per Guidelines - Yes or No (Source) Clinical Relevance Details/Other Notes
N/A N/A N/A N/A N/A N/A N/A

Characteristic Chromosomal or Other Global Mutational Patterns

None

Chromosomal Pattern Molecular Pathogenesis Prevalence -

Common >20%, Recurrent 5-20% or Rare <5% (Disease)

Diagnostic, Prognostic, and Therapeutic Significance - D, P, T Established Clinical Significance Per Guidelines - Yes or No (Source) Clinical Relevance Details/Other Notes
N/A N/A N/A N/A N/A N/A

Gene Mutations (SNV/INDEL)

None

Gene Genetic Alteration Tumor Suppressor Gene, Oncogene, Other Prevalence -

Common >20%, Recurrent 5-20% or Rare <5% (Disease)

Diagnostic, Prognostic, and Therapeutic Significance - D, P, T   Established Clinical Significance Per Guidelines - Yes or No (Source) Clinical Relevance Details/Other Notes
N/A N/A N/A N/A N/A N/A N/A

Note: A more extensive list of mutations can be found in cBioportal, COSMIC, and/or other databases. When applicable, gene-specific pages within the CCGA site directly link to pertinent external content.

Epigenomic Alterations

None

Genes and Main Pathways Involved

None

Gene; Genetic Alteration Pathway Pathophysiologic Outcome
N/A N/A N/A

Genetic Diagnostic Testing Methods

None

Familial Forms

None

Additional Information

None

Links

None

Notes

*Primary authors will typically be those that initially create and complete the content of a page.  If a subsequent user modifies the content and feels the effort put forth is of high enough significance to warrant listing in the authorship section, please contact the Associate Editor or other CCGA representative.  When pages have a major update, the new author will be acknowledged at the beginning of the page, and those who contributed previously will be acknowledged below as a prior author.

Prior Author(s): *Citation of this Page: “Dermal nerve sheath myxoma”. Compendium of Cancer Genome Aberrations (CCGA), Cancer Genomics Consortium (CGC), updated 12/11/2025, https://ccga.io/index.php/STBT5:Dermal nerve sheath myxoma.

References

  1. ↑ Gallager, R. L.; et al. (1980-12). "Neurothekeoma--a benign cutaneous tumor of neural origin". American Journal of Clinical Pathology. 74 (6): 759–764. doi:10.1093/ajcp/74.6.759. ISSN 0002-9173. PMID 7446487. Check date values in: |date= (help)
  2. ↑ Laskin, W. B.; et al. (2000-10). "The "neurothekeoma": immunohistochemical analysis distinguishes the true nerve sheath myxoma from its mimics". Human Pathology. 31 (10): 1230–1241. doi:10.1053/hupa.2000.18474. ISSN 0046-8177. PMID 11070116. Check date values in: |date= (help)
  3. ↑ Fetsch, John F.; et al. (2007-07). "Neurothekeoma: an analysis of 178 tumors with detailed immunohistochemical data and long-term patient follow-up information". The American Journal of Surgical Pathology. 31 (7): 1103–1114. doi:10.1097/PAS.0b013e31802d96af. ISSN 0147-5185. PMID 17592278. Check date values in: |date= (help)
  4. ↑ Fetsch, John F.; et al. (2005-12). "Nerve sheath myxoma: a clinicopathologic and immunohistochemical analysis of 57 morphologically distinctive, S-100 protein- and GFAP-positive, myxoid peripheral nerve sheath tumors with a predilection for the extremities and a high local recurrence rate". The American Journal of Surgical Pathology. 29 (12): 1615–1624. doi:10.1097/01.pas.0000173025.87476.a4. ISSN 0147-5185. PMID 16327434. Check date values in: |date= (help)
  5. ↑ Hornick, Jason L.; et al. (2007-03). "Cellular neurothekeoma: detailed characterization in a series of 133 cases". The American Journal of Surgical Pathology. 31 (3): 329–340. doi:10.1097/01.pas.0000213360.03133.89. ISSN 0147-5185. PMID 17325474. Check date values in: |date= (help)
  6. ↑ Sheth, Sachiv; et al. (2011-03). "Differential gene expression profiles of neurothekeomas and nerve sheath myxomas by microarray analysis". Modern Pathology: An Official Journal of the United States and Canadian Academy of Pathology, Inc. 24 (3): 343–354. doi:10.1038/modpathol.2010.203. ISSN 1530-0285. PMID 21297585. Check date values in: |date= (help)
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