Skip to main content
SpringerLink
Log in

Desmoid Tumor/Deep-Seated Fibromatosis (Desmoid-Type Fibromatosis)

  • Chapter
  • First Online:
Management of Soft Tissue Sarcoma

  • 2437 Accesses

Abstract

Desmoids are enigmatic clonal malignancies of myofibroblastic cells that do not have the ability to metastasize, but cause morbidity and occasionally mortality by locally aggressive growth. They are sometimes termed deep fibromatoses, to distinguish them from superficial fibromatoses such as Dupuytren contracture, trigger finger, or Peyronie disease.

This is a preview of subscription content, log in via an institution to check access.

Access this chapter

Log in via an institution
Chapter
USD 29.95
Price excludes VAT (USA)
  • Available as PDF
  • Read on any device
  • Instant download
  • Own it forever
eBook
USD 139.00
Price excludes VAT (USA)
  • Available as EPUB and PDF
  • Read on any device
  • Instant download
  • Own it forever
Softcover Book
USD 179.99
Price excludes VAT (USA)
  • Compact, lightweight edition
  • Dispatched in 3 to 5 business days
  • Free shipping worldwide - see info
Hardcover Book
USD 199.99
Price excludes VAT (USA)
  • Durable hardcover edition
  • Dispatched in 3 to 5 business days
  • Free shipping worldwide - see info

Tax calculation will be finalised at checkout

Purchases are for personal use only

Institutional subscriptions

References

  1. Half E, Bercovich D, Rozen P. Familial adenomatous polyposis. Orphanet J Rare Dis. 2009;4:22.

    Article  PubMed  Google Scholar 

  2. Dumont AG, Rink L, Godwin AK, et al. A nonrandom association of gastrointestinal stromal tumor (GIST) and desmoid tumor (deep fibromatosis): case series of 28 patients. Ann Oncol. 2012;23:1335–40.

    Article  PubMed  Google Scholar 

  3. Smith AJ, Lewis JJ, Merchant NB, et al. Surgical management of intra-abdominal desmoid tumours. Br J Surg. 2000;87(5):608–13.

    Article  PubMed  CAS  Google Scholar 

  4. Merchant NB, Lewis JJ, Woodruff JM, et al. Extremity and trunk desmoid tumors: a multifactorial analysis of outcome. Cancer. 1999;86(10):2045–52.

    Article  PubMed  CAS  Google Scholar 

  5. Sundaram M, Duffrin H, McGuire MH, et al. Synchronous multicentric desmoid tumors (aggressive fibromatosis) of the extremities. Skeletal Radiol. 1988;17(1):16–9.

    Article  PubMed  CAS  Google Scholar 

  6. Sundaram M, McGuire MH, Herbold DR. Magnetic resonance imaging of soft tissue masses: an evaluation of fifty-three histologically proven tumors. Magn Reson Imaging. 1988;6(3):237–48.

    Article  PubMed  CAS  Google Scholar 

  7. Lazar AJ, Tuvin D, Hajibashi S, et al. Specific mutations in the beta-catenin gene (CTNNB1) correlate with local recurrence in sporadic desmoid tumors. Am J Pathol. 2008;173(5):1518–27.

    Article  PubMed  CAS  Google Scholar 

  8. Davies DR, Armstrong JG, Thakker N, et al. Severe Gardner syndrome in families with mutations restricted to a specific region of the APC gene. Am J Hum Genet. 1995;57(5):1151–8.

    PubMed  CAS  Google Scholar 

  9. Caspari R, Olschwang S, Friedl W, et al. Familial adenomatous polyposis: desmoid tumours and lack of ophthalmic lesions (CHRPE) associated with APC mutations beyond codon 1444. Hum Mol Genet. 1995;4(3):337–40.

    Article  PubMed  CAS  Google Scholar 

  10. Domont J, Benard J, Lacroix L, et al. Detection of β-catenin mutations in primary extra-abdominal fibromatosis (EAF): an ancillary diagnostic tool. J Clin Oncol (Meet Abstr). 2008;26 Suppl 15:10518.

    Google Scholar 

  11. Lazar AJ, Hajibashi S, Lev D. Desmoid tumor: from surgical extirpation to molecular dissection. Curr Opin Oncol. 2009;21(4):352–9.

    Article  PubMed  CAS  Google Scholar 

  12. Alman BA, Li C, Pajerski ME, et al. Increased beta-catenin protein and somatic APC mutations in sporadic aggressive fibromatoses (desmoid tumors). Am J Pathol. 1997;151(2):329–34.

    PubMed  CAS  Google Scholar 

  13. Carlson JW, Fletcher CD. Immunohistochemistry for beta-catenin in the differential diagnosis of spindle cell lesions: analysis of a series and review of the literature. Histopathology. 2007;51(4):509–14.

    Article  PubMed  CAS  Google Scholar 

  14. Deyrup AT, Tretiakova M, Montag AG. Estrogen receptor-beta expression in extraabdominal fibromatoses: an analysis of 40 cases. Cancer. 2006;106(1):208–13.

    Article  PubMed  CAS  Google Scholar 

  15. Rock MG, Pritchard DJ, Reiman HM, et al. Extra-abdominal desmoid tumors. J Bone Joint Surg Am. 1984;66(9):1369–74.

    PubMed  CAS  Google Scholar 

  16. Posner MC, Shiu MH, Newsome JL, et al. The desmoid tumor. Not a benign disease. Arch Surg. 1989;124(2):191–6.

    Article  PubMed  CAS  Google Scholar 

  17. Gaposchkin CG, Bilsky MH, Ginsberg R, et al. Function-sparing surgery for desmoid tumors and other low-grade fibrosarcomas involving the brachial plexus. Neurosurgery. 1998;42(6):1297–301. discussion 1301–1293.

    Article  PubMed  CAS  Google Scholar 

  18. Lewis JJ, Boland PJ, Leung DH, et al. The enigma of desmoid tumors. Ann Surg. 1999;229(6):866–72. discussion 872–863.

    Article  PubMed  CAS  Google Scholar 

  19. Pires de Camargo V, Keohan ML, D’Adamo DR, et al. Clinical outcomes of systemic therapy for patients with deep fibromatosis (desmoid tumor). Cancer. 2010;116(9):2258–65.

    Google Scholar 

  20. Patel SR, Evans HL, Benjamin RS. Combination chemotherapy in adult desmoid tumors. Cancer. 1993;72(11):3244–7.

    Article  PubMed  CAS  Google Scholar 

  21. Patel SR, Benjamin RS. Desmoid tumors respond to chemotherapy: defying the dogma in oncology. J Clin Oncol. 2006;24(1):11–2.

    Article  PubMed  Google Scholar 

  22. Fong Y, Rosen PP, Brennan MF. Multifocal desmoids. Surgery. 1993;114(5):902–6.

    PubMed  CAS  Google Scholar 

  23. Fiore M, Rimareix F, Mariani L, et al. Desmoid-type fibromatosis: a front-line conservative approach to select patients for surgical treatment. Ann Surg Oncol. 2009;16(9):2587–93.

    Article  PubMed  Google Scholar 

  24. Bonvalot S, Eldweny H, Haddad V, et al. Extra-abdominal primary fibromatosis: aggressive management could be avoided in a subgroup of patients. Eur J Surg Oncol. 2008;34(4):462–8.

    Article  PubMed  CAS  Google Scholar 

  25. Tryphonopoulos P, Weppler D, Levi DM, et al. Transplantation for the treatment of intra-abdominal fibromatosis. Transplant Proc. 2005;37(2):1379–80.

    Article  PubMed  CAS  Google Scholar 

  26. Bus PJ, Verspaget HW, van Krieken JH, et al. Treatment of mesenteric desmoid tumours with the anti-oestrogenic agent toremifene: case histories and an overview of the literature. Eur J Gastroenterol Hepatol. 1999;11(10):1179–83.

    Article  PubMed  CAS  Google Scholar 

  27. Kinzbrunner B, Ritter S, Domingo J, et al. Remission of rapidly growing desmoid tumors after tamoxifen therapy. Cancer. 1983;52(12):2201–4.

    Article  PubMed  CAS  Google Scholar 

  28. Meazza C, Bisogno G, Gronchi A, et al. Aggressive fibromatosis in children and adolescents: the Italian experience. Cancer. 2010;116(1):233–40.

    PubMed  CAS  Google Scholar 

  29. Bertagnolli MM, Morgan JA, Fletcher CD, et al. Multimodality treatment of mesenteric desmoid tumours. Eur J Cancer. 2008;44(16):2404–10.

    Article  PubMed  Google Scholar 

  30. Weiss AJ, Horowitz S, Lackman RD. Therapy of desmoid tumors and fibromatosis using vinorelbine. Am J Clin Oncol. 1999;22(2):193–5.

    Article  PubMed  CAS  Google Scholar 

  31. Meazza C, Casanova M, Trecate G, et al. Objective response to hydroxyurea in a patient with heavily pre-treated aggressive fibromatosis. Pediatr Blood Cancer. 2010;55(3):587–8.

    Article  PubMed  Google Scholar 

  32. Garbay D, Le Cesne A, Penel N, et al. Chemotherapy in patients with desmoid tumors: a study from the French Sarcoma Group (FSG). Ann Oncol. 2012;23(1):182–6.

    Article  PubMed  CAS  Google Scholar 

  33. Lev D, Kotilingam D, Wei C, et al. Optimizing treatment of desmoid tumors. J clin oncol: official J Am Soc Clin Oncol. 2007;25(13):1785–91.

    Article  Google Scholar 

  34. Chugh R, Wathen JK, Patel SR, et al. Efficacy of imatinib in aggressive fibromatosis: results of a phase II multicenter Sarcoma Alliance for Research through Collaboration (SARC) trial. Clin Cancer Res. 2010;16(19):4884–91.

    Article  PubMed  CAS  Google Scholar 

  35. Penel N, Le Cesne A, Bui BN, et al. Imatinib for progressive and recurrent aggressive fibromatosis (desmoid tumors): an FNCLCC/French Sarcoma Group phase II trial with a long-term follow-up. Ann Oncol. 2011;22:452–7.

    Article  PubMed  Google Scholar 

  36. de Camargo VP, Keohan ML, D’Adamo DR, et al. Clinical outcomes of systemic therapy for patients with deep fibromatosis (desmoid tumor). Cancer. 2010;116(9):2258–65.

    PubMed  Google Scholar 

  37. Heinrich MC, Joensuu H, Demetri GD, et al. Phase II, open-label study evaluating the activity of imatinib in treating life-threatening malignancies known to be associated with imatinib-sensitive tyrosine kinases. Clin Cancer Res. 2008;14(9):2717–25.

    Article  PubMed  CAS  Google Scholar 

  38. Skubitz KM, Manivel JC, Clohisy DR, et al. Response of imatinib-resistant extra-abdominal aggressive fibromatosis to sunitinib: case report and review of the literature on response to tyrosine kinase inhibitors. Cancer Chemother Pharmacol. 2009;64(3):635–40.

    Article  PubMed  CAS  Google Scholar 

  39. Gounder M, Lefkowitz RA, Keohan ML, et al. Activity of sorafenib against desmoid tumor/deep fibromatoses. Clin Cancer Res. 2011;17:4082–90.

    Article  PubMed  CAS  Google Scholar 

  40. Hosalkar HS, Fox EJ, Delaney T, et al. Desmoid tumors and current status of management. Orthop Clin North Am. 2006;37(1):53–63.

    Article  PubMed  Google Scholar 

  41. Seinfeld J, Kleinschmidt-Demasters BK, Tayal S, et al. Desmoid-type fibromatoses involving the brachial plexus: treatment options and assessment of c-KIT mutational status. J Neurosurg. 2006;104(5):749–56.

    Article  PubMed  CAS  Google Scholar 

  42. Salas S, Dufresne A, Bui B, et al. Prognostic factors influencing progression-free survival determined from a series of sporadic desmoid tumors: a wait-and-see policy according to tumor presentation. J Clin Oncol. 2011;29(26):3553–8.

    Article  PubMed  Google Scholar 

  43. Crago AM, Denton B, Mezhir JJ, et al. A prognostic nomogram for prediction of recurrence in desmoid fibromatosis. Ann Surg. (2012, submitted).

    Google Scholar 

  44. Ballo MT, Zagars GK, Pollack A, et al. Desmoid tumor: prognostic factors and outcome after surgery, radiation therapy, or combined surgery and radiation therapy. J Clin Oncol. 1999;17(1):158–67.

    PubMed  CAS  Google Scholar 

  45. Gronchi A, Casali PG, Mariani L, et al. Quality of surgery and outcome in extra-abdominal aggressive fibromatosis: a series of patients surgically treated at a single institution. J Clin Oncol. 2003;21(7):1390–7.

    Article  PubMed  CAS  Google Scholar 

  46. Stoeckle E, Coindre JM, Longy M, et al. A critical analysis of treatment strategies in desmoid tumours: a review of a series of 106 cases. Eur J Surg Oncol. 2009;35(2):129–34.

    Article  PubMed  CAS  Google Scholar 

Download references

Author information

Authors and Affiliations

  1. International Center & Department of Surgery, Memorial Sloan-Kettering Cancer Center, New York, NY, USA

    Murray F. Brennan

  2. Department of Pathology, Memorial Sloan-Kettering Cancer Center, New York, NY, USA

    Cristina R. Antonescu

  3. Departments of Medicine Pediatrics, and Orthopaedics, Mount Sinai School of Medicine, New York, NY, USA

    Robert G. Maki

Authors
  1. Murray F. Brennan
    View author publications

    You can also search for this author in PubMed Google Scholar

  2. Cristina R. Antonescu
    View author publications

    You can also search for this author in PubMed Google Scholar

  3. Robert G. Maki
    View author publications

    You can also search for this author in PubMed Google Scholar

Rights and permissions

Reprints and permissions

Copyright information

© 2013 Springer Science+Business Media New York

About this chapter

Cite this chapter

Brennan, M.F., Antonescu, C.R., Maki, R.G. (2013). Desmoid Tumor/Deep-Seated Fibromatosis (Desmoid-Type Fibromatosis). In: Management of Soft Tissue Sarcoma. Springer, New York, NY. https://doi.org/10.1007/978-1-4614-5004-7_10

Download citation

  • DOI: https://doi.org/10.1007/978-1-4614-5004-7_10

  • Published:

  • Publisher Name: Springer, New York, NY

  • Print ISBN: 978-1-4614-5003-0

  • Online ISBN: 978-1-4614-5004-7

  • eBook Packages: MedicineMedicine (R0)

Publish with us

Policies and ethics

Search

Navigation