Skip to main content

Advertisement

Log in

Desmoids: A Revelation in Biology and Treatment

  • Bone and Soft Tissue Sarcomas
  • Published:
Annals of Surgical Oncology Aims and scope Submit manuscript

Abstract

Introduction

Using a preoperative neoadjuvant chemoradiation protocol, followed by complete excision, we have achieved local control rates exceeding that found in most large series.

Methods

From October 1990 through May 2008, resectable desmoids were initially treated with a preoperative protocol using Adriamycin 30 mg × 3 days continuous intravenous infusion followed by 3,000 cGy of radiation (300 cGy fractions over 10 days). Resection was performed 4–6 weeks later. After 2001, all patients were initially offered Tamoxifen 120 mg/day and Celebrex 400 mg/day for 1 year. Patients who progressed on Tamox/Celeb were treated with protocol and those with stabilization or regression were observed. Patient demographics, tumor size, history of previous recurrences, and follow-up status were recorded prospectively.

Results

There were 40 females and 12 males with a mean follow-up of 45 months. Forty patients presented with primary tumors and eight presented with recurrent disease. Thirty-nine patients had surgical resection and 13 were observed. Thirty patients underwent the neoadjuvant protocol. Tamoxifen and Celebrex were used in 16 patients, 6 had stabilization in growth, 1 had a 50% reduction in the size of the tumor, there was 1 complete regression, and 8 progressed. Of the patients who had resectable disease Tamoxifen and Celebrex obviated surgery in 30%. Overall 13% (5) of patients developed a recurrence. There were three recurrences among the protocol group for a local control rate of 90%.

Conclusion

Although our neoadjuvant protocol demonstrates the best results to date in eradication of disease, an initial conservative approach is reasonable to determine who would most benefit from surgery.

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

Access this article

Price excludes VAT (USA)
Tax calculation will be finalised during checkout.

Instant access to the full article PDF.

Fig. 1
Fig. 2
Fig. 3

Similar content being viewed by others

References

  1. Weiss SW, Goldblum JR, Enzinger FM. Enzinger and Weiss’ soft tissue tumors. 5th ed. Philadelphia: Mosby Elsevier; 2008.

    Google Scholar 

  2. Enzinger FM, Shiraki M. Musculo-aponeurotic fibromatosis of the shoulder girdle (extra-abdominal desmoid). Analysis of thirty cases followed up for ten or more years. Cancer. 1967;20(7):1131–40.

    Article  PubMed  CAS  Google Scholar 

  3. Karakousis CP, Mayordomo J, Zografos GC, Driscoll DL. Desmoid tumors of the trunk and extremity. Cancer. 1993;72(5):1637–41.

    Article  PubMed  CAS  Google Scholar 

  4. Miralbell R, Suit HD, Mankin HJ, et al. Fibromatoses: from postsurgical surveillance to combined surgery and radiation therapy. Int J Radiat Oncol Biol Phys. 1990;18(3):535–40.

    PubMed  CAS  Google Scholar 

  5. Pritchard DJ, Nascimento AG, Petersen IA. Local control of extra-abdominal desmoid tumors. J Bone Joint Surg Am. 1996;78(6):848–54.

    PubMed  CAS  Google Scholar 

  6. 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 

  7. Baliski CR, Temple WJ, Arthur K, Schachar NS. Desmoid tumors: a novel approach for local control. J Surg Oncol. 2002;80(2):96–9.

    Article  PubMed  Google Scholar 

  8. Mack LA, Crowe PJ, Yang JL, et al. Preoperative chemoradiotherapy (modified Eilber protocol) provides maximum local control and minimal morbidity in patients with soft tissue sarcoma. Ann Surg Oncol. 2005;12(8):646–53.

    Article  PubMed  Google Scholar 

  9. Bridge JA, Swarts SJ, Buresh C, et al. Trisomies 8 and 20 characterize a subgroup of benign fibrous lesions arising in both soft tissue and bone. Am J Pathol. 1999;154(3):729–33.

    PubMed  CAS  Google Scholar 

  10. De Wever I, Dal Cin P, Fletcher CD, et al. Cytogenetic, clinical, and morphologic correlations in 78 cases of fibromatosis: a report from the CHAMP Study Group. CHromosomes And Morphology. Mod Pathol. 2000;13(10):1080–5.

    Article  PubMed  Google Scholar 

  11. Tejpar S, Nollet F, Li C, et al. Predominance of beta-catenin mutations and beta-catenin dysregulation in sporadic aggressive fibromatosis (desmoid tumor). Oncogene. 1999;18(47):6615–20.

    Article  PubMed  CAS  Google Scholar 

  12. 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 

  13. Lewis JJ, Boland PJ, Leung DH, et al. The enigma of desmoid tumors. Ann Surg. 1999;229(6):866–72; discussion 872–3.

    Article  PubMed  CAS  Google Scholar 

  14. Janinis J, Patriki M, Vini L, et al. The pharmacological treatment of aggressive fibromatosis: a systematic review. Ann Oncol. 2003;14(2):181–90.

    Article  PubMed  CAS  Google Scholar 

  15. Lim CL, Walker MJ, Mehta RR, Das Gupta TK. Estrogen and antiestrogen binding sites in desmoid tumors. Eur J Cancer Clin Oncol. 1986;22(5):583–7.

    Article  PubMed  CAS  Google Scholar 

  16. 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 

  17. Lev D, Kotilingam D, Wei C, et al. Optimizing treatment of desmoid tumors. J Clin Oncol. 2007;25(13):1785–91.

    Article  PubMed  Google Scholar 

  18. 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 

  19. Spear MA, Jennings LC, Mankin HJ, et al. Individualizing management of aggressive fibromatoses. Int J Radiat Oncol Biol Phys. 1998;40(3):637–45.

    PubMed  CAS  Google Scholar 

  20. Leithner A, Gapp M, Leithner K, et al. Margins in extra-abdominal desmoid tumors: a comparative analysis. J Surg Oncol. 2004;86(3):152–6.

    Article  PubMed  Google Scholar 

  21. 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.

    PubMed  CAS  Google Scholar 

Download references

Author information

Authors and Affiliations

Authors

Corresponding author

Correspondence to Walley J. Temple MD, FRCSC, FACS.

Rights and permissions

Reprints and permissions

About this article

Cite this article

Francis, W.P., Zippel, D., Mack, L.A. et al. Desmoids: A Revelation in Biology and Treatment. Ann Surg Oncol 16, 1650–1654 (2009). https://doi.org/10.1245/s10434-008-0285-4

Download citation

  • Received:

  • Revised:

  • Accepted:

  • Published:

  • Issue Date:

  • DOI: https://doi.org/10.1245/s10434-008-0285-4

Keywords

Navigation