Palmar fibromatosis and plantar fibromatosis
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 | Fibroblastic and myofibroblastic tumours |
| Type | Palmar fibromatosis and plantar fibromatosis |
| Subtype(s) | N/A |
Related Terminology
| Acceptable | Ledderhose disease |
| Not Recommended | N/A |
Other: Plantar fibromatosis
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
Although studies suggest that activation of the WNT/beta-catenin signaling pathway may be present on the basis of immunohistochemistry, genetic drivers (e.g. CTNNB1 or APC) have yet to be identified.[1][2][3][4][5]
| Gene; Genetic Alteration | Pathway | Pathophysiologic Outcome |
|---|---|---|
| N/A | N/A | N/A |
Genetic Diagnostic Testing Methods
Not applicable
Familial Forms
None
Additional Information
None
Links
None
References
- ↑ Montgomery, E.; et al. (2001-07). "Superficial fibromatoses are genetically distinct from deep fibromatoses". Modern Pathology: An Official Journal of the United States and Canadian Academy of Pathology, Inc. 14 (7): 695–701. doi:10.1038/modpathol.3880374. ISSN 0893-3952. PMID 11455002. Check date values in:
|date=(help) - ↑ Dolmans, Guido H.; et al. (2011-07-28). "Wnt signaling and Dupuytren's disease". The New England Journal of Medicine. 365 (4): 307–317. doi:10.1056/NEJMoa1101029. ISSN 1533-4406. PMID 21732829.
- ↑ Michou, Laëtitia; et al. (2012-01). "Genetics of Dupuytren's disease". Joint Bone Spine. 79 (1): 7–12. doi:10.1016/j.jbspin.2011.05.027. ISSN 1778-7254. PMID 21803632. Check date values in:
|date=(help) - ↑ Mosakhani, Neda; et al. (2010-11). "Unique microRNA profile in Dupuytren's contracture supports deregulation of β-catenin pathway". Modern Pathology: An Official Journal of the United States and Canadian Academy of Pathology, Inc. 23 (11): 1544–1552. doi:10.1038/modpathol.2010.146. ISSN 1530-0285. PMID 20676061. Check date values in:
|date=(help) - ↑ Stewart, Brian D.; et al. (2021-07). "Palmar and plantar fibromatosis: a review". Journal of Pathology and Translational Medicine. 55 (4): 265–270. doi:10.4132/jptm.2021.06.14. ISSN 2383-7837. PMC 8353138 Check
|pmc=value (help). PMID 34225446 Check|pmid=value (help). Check date values in:|date=(help)
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: “Palmar fibromatosis and plantar fibromatosis”. Compendium of Cancer Genome Aberrations (CCGA), Cancer Genomics Consortium (CGC), updated 10/22/2025, https://ccga.io/index.php/STBT5:Palmar fibromatosis and plantar fibromatosis.