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Outcome After Surgical Treatment of Dermatofibrosarcoma Protuberans (DFSP): Does it Require Extensive Follow-up and What is an Adequate Resection Margin?

  • Sarcoma
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Abstract

Introduction

Dermatofibrosarcoma protuberans (DFSP) is a rare cutaneous tumour of indeterminate malignant potential. The mainstay treatment for DFSP is surgical resection. Given the reported high local recurrence rate, the ideal resection margin for DFSP is unclear. The purpose of this study was to ascertain the local recurrence and metastatic rate of DFSP and DFSP with fibrosarcomatous degeneration (FS-DFSP), with specific attention to margin status in an attempt to address the issue of margin adequacy.

Methods

Patients treated for DFSP at a single sarcoma centre were identified from a prospective database. DFSP and FS-DFSP patients with and without prior surgery were included. Patients were followed after surgery to monitor complications, local recurrence and metastasis.

Results

The study included 200 patients: 166 patients with DFSP and 34 patients with FS-DFSP. In the DFSP group, nine patients (5.4%) had positive margins, one case (0.6%) developed local recurrence (LR) and no patients developed distant metastases. In the FS-DFSP group, seven patients (20.6%) had positive margins, six patients (17.6%) developed local recurrence (LR) and eight patients (23.5%) developed distant metastases, of which three (37.5%) were in the lungs, one (12.5%) in bone and four (50%) in other soft tissue sites.

Discussion and Conclusion

Local recurrence and metastases are extremely rare in patients with DFSP. Achieving a negative as opposed to a wide surgical margin may be sufficient to avoid local recurrence of most DFSP. We suggest that no ongoing surveillance for local or systemic relapse is required for DFSP patients after negative margin resection. For FS-DFSP, we recommend the same surveillance schedule, based on tumour grade, as other soft tissue sarcoma.

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References

  1. Criscione VD, Weinstock MA. Descriptive epidemiology of dermatofibrosarcoma protuberans in the United States, 1973 to 2002. J Am Acad Dermatol. 2007;56(6):968–73.

    Article  PubMed  Google Scholar 

  2. Mangham DC. World Health Organisation classification of tumours: pathology and genetics of tumours of soft tissue and bone. J Bone Joint Surg Br. 2004;86-B(3):466–466. https://doi.org/10.1302/0301-620X.86B3.0860466b.

    Article  Google Scholar 

  3. Kreicher KL, Kurlander DE, Gittleman HR, Barnholtz-Sloan JS, Bordeaux JS. Incidence and survival of primary dermatofibrosarcoma protuberans in the United States. Dermatologic Surg. 2016;42:S24-31.

    Article  CAS  Google Scholar 

  4. Kuzel P, Metelitsa AI, Dover DC, Salopek TG. Epidemiology of dermatofibrosarcoma protuberans in Alberta, Canada, from 1988 to 2007. Dermatologic Surg. 2012;38(9):1461–8.

    Article  CAS  Google Scholar 

  5. Rouhani P, Fletcher CDM, Devesa SS, Toro JR. Cutaneous soft tissue sarcoma incidence patterns in the US: An analysis of 12,114 cases. Cancer. 2008;113(3):616–27.

    Article  PubMed  Google Scholar 

  6. Dadone-Montaudié B, Alberti L, Duc A, Delespaul L, Lesluyes T, Pérot G, et al. Alternative PDGFD rearrangements in dermatofibrosarcomas protuberans without PDGFB fusions. Mod Pathol. 2018;31(11):1683–93.

    Article  PubMed  Google Scholar 

  7. Dickson BC, Hornick JL, Fletcher CDM, Demicco EG, Howarth DJ, Swanson D, et al. Dermatofibrosarcoma protuberans with a novel COL6A3-PDGFD fusion gene and apparent predilection for breast. Genes Chromosom Cancer. 2018;57(9):437–45.

    Article  CAS  PubMed  Google Scholar 

  8. Farma JM, Ammori JB, Zager JS, Marzban SS, Bui MM, Bichakjian CK, et al. Dermatofibrosarcoma protuberans: How wide should WE resect? Ann Surg Oncol. 2010;17(8):2112–8.

    Article  PubMed  Google Scholar 

  9. Huis in t Veld EA, van Coevorden F, Grünhagen DJ, Smith MJ, van Akkooi ACJ, Wouters MWJM, et al. Outcome after surgical treatment of dermatofibrosarcoma protuberans: Is clinical follow-up always indicated? Cancer. 2019;125(5):735–41.

    Article  PubMed  Google Scholar 

  10. Voth H, Landsberg J, Hinz T, Wenzel J, Bieber T, Reinhard G, et al. Management of dermatofibrosarcoma protuberans with fibrosarcomatous transformation: An evidence-based review of the literature. J Eur Acad Dermatology Venereol. 2011;25(12):1385–91.

    Article  CAS  Google Scholar 

  11. Reha J, Katz SC. Dermatofibrosarcoma Protuberans. Surg Clin North Am. 2016;96(5):1031–46. https://doi.org/10.1016/j.suc.2016.05.006.

    Article  PubMed  Google Scholar 

  12. Stojadinovic A, Karpoff HM, Antonescu CR, Shah JP, Singh B, Spiro RH, et al. Dermatofibrosarcoma protuberans of the head and neck. Ann Surg Oncol. 2000;7(9):696–704.

    Article  CAS  PubMed  Google Scholar 

  13. Rutgers EJ, Kroon BB, Albus-Lutter CE, Gortzak E. Dermatofibrosarcoma protuberans: treatment and prognosis. Eur J Surg Oncol. 1992;18(3):241–8.

    CAS  PubMed  Google Scholar 

  14. Meguerditchian A-N, Wang J, Lema B, Kraybill WG, Zeitouni NC, Kane JM. Wide excision or Mohs micrographic surgery for the treatment of primary dermatofibrosarcoma protuberans. Am J Clin Oncol. 2010;33(3):300–3.

    Article  PubMed  Google Scholar 

  15. Paradisi A, Abeni D, Rusciani A, Cigna E, Wolter M, Scuderi N, et al. Dermatofibrosarcoma protuberans: Wide local excision vs. Mohs micrographic surgery. Cancer Treat Rev. 2008;34(8):728–36. https://doi.org/10.1016/j.ctrv.2008.06.002.

    Article  CAS  PubMed  Google Scholar 

  16. Fields RC, Hameed M, Qin L-X, Moraco N, Jia X, Maki RG, et al. Dermatofibrosarcoma protuberans (DFSP): Predictors of Recurrence and the Use of Systemic Therapy. Ann Surg Oncol. 2011;18(2):328–36. https://doi.org/10.1245/s10434-010-1316-5.

    Article  PubMed  Google Scholar 

  17. Bichakjian CK, Olencki T, Alam M, Andersen JS, Berg D, Bowen GM, et al. Dermatofibrosarcoma Protuberans. Featured Updates to the NCCN Guidelines. 2014;12(6):863–838.

    Google Scholar 

  18. Saiag P, Grob JJ, Lebbe C, Malvehy J, Del Marmol V, Pehamberger H, et al. Diagnosis and treatment of dermatofibrosarcoma protuberans European consensus-based interdisciplinary guideline. Eur J Cancer. 2015;51(17):2604–8. https://doi.org/10.1016/j.ejca.2015.06.108.

    Article  PubMed  Google Scholar 

  19. Gronchi A, Miceli R, Fiore M, Collini P, Lozza L, Grosso F, et al. Extremity soft tissue sarcoma: Adding to the prognostic meaning of local failure. Ann Surg Oncol. 2007;14(5):1583–90.

    Article  PubMed  Google Scholar 

  20. Kainhofer V, Smolle MA, Szkandera J, Liegl-Atzwanger B, Maurer-Ertl W, Gerger A, et al. The width of resection margins influences local recurrence in soft tissue sarcoma patients. Eur J Surg Oncol. 2016;42(6):899–906. https://doi.org/10.1016/j.ejso.2016.03.026.

    Article  CAS  PubMed  Google Scholar 

  21. Gundle KR, Kafchinski L, Gupta S, Griffin AM, Dickson BC, Chung PW, et al. Analysis of margin classification systems for assessing the risk of local recurrence after soft tissue sarcoma resection. J Clin Oncol. 2018;36(7):704–9.

    Article  PubMed  Google Scholar 

  22. O’Sullivan B, Davis AM, Turcotte R, Bell R, Catton C, Chabot P, et al. Preoperative versus postoperative radiotherapy in soft-tissue sarcoma of the limbs: a randomised trial. Lancet. 2002;359(9325):2235–41.

    Article  PubMed  Google Scholar 

  23. Wilson DAJ, Gazendam A, Visgauss J, Perrin D, Griffin AM, Chung PW, et al. Designing a Rational Follow-Up Schedule for Patients with Extremity Soft Tissue Sarcoma. Ann Surg Oncol. 2020;27(6):2033–41.

    Article  PubMed  Google Scholar 

  24. Hao X, Billings SD, Wu F, Stultz TW, Procop GW, Mirkin G, et al. Dermatofibrosarcoma protuberans: Update on the diagnosis and treatment. J Clin Med. 2020;9(6):1752.

    Article  CAS  PubMed  PubMed Central  Google Scholar 

  25. Castle KO, Guadagnolo BA, Tsai CJ, Feig BW, Zagars GK. Dermatofibrosarcoma protuberans: Long-term outcomes of 53 patients treated with conservative surgery and radiation therapy. Int J Radiat Oncol Biol Phys. 2013;86(3):585–90. https://doi.org/10.1016/j.ijrobp.2013.02.024.

    Article  PubMed  Google Scholar 

  26. Liu M, Wang X, Cai L, Liu H, Chen E, Cui N. Prognostic factors and treatment of 74 patients with dermatofibro-sarcoma protuberans. Zhonghua Zhong Liu Za Zhi. 2005;27(2):122–5.

    PubMed  Google Scholar 

  27. Odueyungbo M, Ratner D. Update on the use and treatment of targeted molecular inhibitors for locally advanced and metastatic non-melanoma skin cancers. Dermatologic Surg. 2016;42:S49-56.

    Article  CAS  Google Scholar 

  28. Rutkowski P, Klimczak A, Ługowska I, Jagielska B, Wągrodzki M, Dębiec-Rychter M, et al. Long-term results of treatment of advanced dermatofibrosarcoma protuberans (DFSP) with imatinib mesylate — The impact of fibrosarcomatous transformation. Eur J Surg Oncol. 2017;43(6):1134–41. https://doi.org/10.1016/j.ejso.2017.03.011.

    Article  CAS  PubMed  Google Scholar 

  29. Navarrete-Dechent C, Mori S, Barker CA, Dickson MA, Nehal KS. Imatinib Treatment for Locally Advanced or Metastatic Dermatofibrosarcoma Protuberans: A Systematic Review. JAMA Dermatology. 2019;155(3):361–9.

    Article  PubMed  PubMed Central  Google Scholar 

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Authors and Affiliations

  1. Department of Ortopaedics, College of Medicine, King Saud University, Riyadh, Saudi Arabia

    Ibrahim Alshaygy MD, MHSc

  2. College of Medicine, Alfaisal University, Riyadh, Saudi Arabia

    Ibrahim Alshaygy MD, MHSc

  3. Orthopédie, Hospital de la Timone, Provence-Alpes-Côte d’Azu, Marseille, France

    Jean-Camille Mattei MD

  4. Orthopedic Surgery, Universite de Montreal, Montreal, QC, Canada

    Georges Basile MD, FRCSC

  5. Division of Orthopaedic Surgery, Mount Sinai Hospital, Toronto, ON, Canada

    Anthony M. Griffin MSc, Jay S. Wunder MD, FRCSC & Peter C. Ferguson MD, FRCSC

  6. Department of Surgery, University of Toronto, Toronto, ON, Canada

    Anthony M. Griffin MSc, Rebecca A. Gladdy MD, FRCSC, Carol J. Swallow MD, FRCSC, Jay S. Wunder MD, FRCSC & Peter C. Ferguson MD, FRCSC

  7. Division of General Surgery, Mount Sinai Hospital, Toronto, ON, Canada

    Rebecca A. Gladdy MD, FRCSC & Carol J. Swallow MD, FRCSC

  8. Department of Pathology and Laboratory Medicine, Mount Sinai Hospital, University of Toronto, Toronto, ON, Canada

    Brendan C. Dickson MD, FRCPC

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  1. Ibrahim Alshaygy MD, MHSc
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  2. Jean-Camille Mattei MD
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  3. Georges Basile MD, FRCSC
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  5. Rebecca A. Gladdy MD, FRCSC
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Correspondence to Ibrahim Alshaygy MD, MHSc.

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Alshaygy, I., Mattei, JC., Basile, G. et al. Outcome After Surgical Treatment of Dermatofibrosarcoma Protuberans (DFSP): Does it Require Extensive Follow-up and What is an Adequate Resection Margin?. Ann Surg Oncol 30, 3106–3113 (2023). https://doi.org/10.1245/s10434-022-12953-8

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