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Chest Wall Fibromatosis

MedGen UID:
232051
•Concept ID:
C1332930
•
Neoplastic Process
Synonyms: Chest Wall Desmoid Neoplasm; Chest Wall Desmoid Tumor; Desmoid Neoplasm of Chest Wall; Desmoid Neoplasm of the Chest Wall; Desmoid Tumor of Chest Wall; Desmoid Tumor of the Chest Wall

Definition

An extraabdominal fibromatosis arising from the soft tissue of the chest wall. It is characterized by the presence of elongated spindle-shaped fibroblasts, collagenous stroma formation, and an infiltrative growth pattern. [from NCI]

Term Hierarchy

CClinical test,  RResearch test,  OOMIM,  GGeneReviews,  VClinVar  
  • CROGVChest Wall Fibromatosis

Professional guidelines

PubMed

Evolution of Initial Treatment for Desmoid Tumors.
Bartholomew AJ, Rhodin KE, Noteware L, Moris D, Kanu E, Masoud S, Howell TC, Burner D, Kim CY, Nussbaum DP, Zani S, Lidsky ME, Allen PJ, Riedel RF, Blazer DG 3rd
Ann Surg Oncol 2024 Nov;31(12):7943-7949. Epub 2024 Aug 12 doi: 10.1245/s10434-024-15938-x. PMID: 39133446
Radiotherapy in the treatment of aggressive fibromatosis: experience from a single institution.
Seidensaal K, Harrabi SB, Weykamp F, Herfarth K, Welzel T, Mechtersheimer G, Lehner B, Schneider M, Fröhling S, Egerer G, Debus J, Uhl M
Radiat Oncol 2020 Jun 5;15(1):143. doi: 10.1186/s13014-020-01565-9. PMID: 32503580Free PMC Article
Elastofibroma dorsi--differential diagnosis in chest wall tumours.
Daigeler A, Vogt PM, Busch K, Pennekamp W, Weyhe D, Lehnhardt M, Steinstraesser L, Steinau HU, Kuhnen C
World J Surg Oncol 2007 Feb 5;5:15. doi: 10.1186/1477-7819-5-15. PMID: 17280612Free PMC Article

Recent clinical studies

Therapy

Juvenile female with chest wall fibromatosis located posteriorly to the right breast: radiation therapy or wait and watch?
Akhavan A, Binesh F, Kargar K, Navabii H
BMJ Case Rep 2013 Feb 20;2013 doi: 10.1136/bcr-2012-006355. PMID: 23429011Free PMC Article
Sirolimus therapy for fibromatosis and multifocal renal cell carcinoma in a child with tuberous sclerosis complex.
Pressey JG, Wright JM, Geller JI, Joseph DB, Pressey CS, Kelly DR
Pediatr Blood Cancer 2010 Jul 1;54(7):1035-7. doi: 10.1002/pbc.22401. PMID: 20108343
Chest wall fibromatosis associated with silicone breast implants.
Aaron AD, O'Mara JW, Legendre KE, Evans SR, Attinger CE, Montgomery EA
Surg Oncol 1996 Apr;5(2):93-9. doi: 10.1016/s0960-7404(96)80006-5. PMID: 8853244

Prognosis

Juvenile female with chest wall fibromatosis located posteriorly to the right breast: radiation therapy or wait and watch?
Akhavan A, Binesh F, Kargar K, Navabii H
BMJ Case Rep 2013 Feb 20;2013 doi: 10.1136/bcr-2012-006355. PMID: 23429011Free PMC Article

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