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Surgical Outcomes of Primary Dermatofibrosarcoma Protuberans: A Retrospective, Multicenter Study

  • Sarcoma
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Abstract

Background

Dermatofibrosarcoma protuberans (DFSP) is a locally aggressive tumor with a low rate of metastatic disease. Previous series have shown a superiority of Mohs micrographic surgery (MMS) compared with wide local excision (WLE). Likewise, there is paucity of data examining the long-term follow-up of patients.

Objective

The purpose of the current study was to examine the outcome of surgical treatment of primary DFSP of the trunk and extremities.

Methods

We reviewed 236 patients (115 females, 121 males, mean age 41 ± 15 years) undergoing MMS (n = 81, 34%) or WLE (n = 155, 66%) to treat a primary DFSP. Mean tumor size and follow-up was 4 ± 2 cm and 7 years, respectively. Final margins were negative in 230 (97%) patients.

Results

There was no difference (p > 0.05) in patient age, sex, tumor size, negative margin excision, or history of a previous inadvertent excision between patients who underwent WLE and those undergoing MMS. There were two cases of local recurrence and two cases of metastasis, with no difference in the 5-year local recurrence-free survival (98% vs. 99%, p = 0.69) or metastatic-free survival (98% vs. 100%, p= 0.27) between WLE and MMS.

Conclusion

There was no difference in oncologic outcome comparing MMS with WLE for DFSP outside the head and neck. The goal of treatment for DFSP is to achieve a negative margin, regardless of surgical treatment modalities. A ‘less is more’ approach to follow-up can likely be taken for patients with completely resected DFSP in easy-to-examine anatomical areas. In these patients, no formal follow-up should be required.

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References

  1. Huis In ‘t Veld EA, van Coevorden F, Grunhagen DJ, 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 

  2. Rouhani P, Fletcher CD, Devesa SS, Toro JR. Cutaneous soft tissue sarcoma incidence patterns in the U.S.: an analysis of 12,114 cases. Cancer. 2008;113(3):616–27.

    Article  PubMed  Google Scholar 

  3. Liang CA, Jambusaria-Pahlajani A, Karia PS, Elenitsas R, Zhang PD, Schmults CD. A systematic review of outcome data for dermatofibrosarcoma protuberans with and without fibrosarcomatous change. J Am Acad Dermatol. 2014;71(4):781–6.

    Article  PubMed  Google Scholar 

  4. Saiag P, Grob JJ, Lebbe C, et al. Diagnosis and treatment of dermatofibrosarcoma protuberans European consensus-based interdisciplinary guideline. Eur J Cancer. 2015;51(17):2604–8.

    Article  PubMed  Google Scholar 

  5. Foroozan M, Sei JF, Amini M, Beauchet A, Saiag P. Efficacy of Mohs micrographic surgery for the treatment of dermatofibrosarcoma protuberans: systematic review. Arch Dermatol. 2012;148(9):1055–63.

    Article  PubMed  Google Scholar 

  6. Lowe GC, Onajin O, Baum CL, et al. A comparison of mohs micrographic surgery and wide local excision for treatment of dermatofibrosarcoma protuberans with long-term follow-up: the mayo clinic experience. Dermatol Surg. 2017;43(1):98–106.

    Article  CAS  PubMed  Google Scholar 

  7. van Lee CB, Kan WC, Gran S, et al. Dermatofibrosarcoma protuberans re-excision and recurrence rates in the Netherlands between 1989 and 2016. Acta Derm Venereol. 2019;99(12):1160–5.

    PubMed  Google Scholar 

  8. Gloster HM Jr, Harris KR, Roenigk RK. A comparison between Mohs micrographic surgery and wide surgical excision for the treatment of dermatofibrosarcoma protuberans. J Am Acad Dermatol. 1996;35(1):82–7.

    PubMed  Google Scholar 

  9. Huis In’t Veld EA, Grunhagen DJ, van Coevorden F, et al. Adequate surgical margins for dermatofibrosarcoma protuberans: a multi-centre analysis. Eur J Surg Oncol. 2021;47(2):436–42.

    Article  Google Scholar 

  10. Gundle KR, Kafchinski L, Gupta S, 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 

  11. Monnier D, Vidal C, Martin L, et al. Dermatofibrosarcoma protuberans: a population-based cancer registry descriptive study of 66 consecutive cases diagnosed between 1982 and 2002. J Eur Acad Dermatol Venereol. 2006;20(10):1237–42.

    Article  CAS  PubMed  Google Scholar 

  12. Bogucki B, Neuhaus I, Hurst EA. Dermatofibrosarcoma protuberans: a review of the literature. Dermatol Surg. 2012;38(4):537–51.

    Article  CAS  PubMed  Google Scholar 

  13. Lindner NJ, Scarborough MT, Powell GJ, Spanier S, Enneking WF. Revision surgery in dermatofibrosarcoma protuberans of the trunk and extremities. Eur J Surg Oncol. 1999;25(4):392–7.

    Article  CAS  PubMed  Google Scholar 

  14. Mallett KE, Almubarak S, Claxton RM, et al. Preoperative Risk Factors for Fibrosarcomatous Transformation in Dermatofibrosarcoma Protuberans. Anticancer Res. 2022;42(1):105–8.

    Article  PubMed  Google Scholar 

  15. Gladdy RA, Wunder JS. Risk-stratified surveillance in dermatofibrosarcoma protuberans: Less is more. Cancer. 2019;125(5):670–2.

    Article  PubMed  Google Scholar 

  16. Akram J, Wooler G, Lock-Andersen J. Dermatofibrosarcoma protuberans: clinical series, national Danish incidence data and suggested guidelines. J Plast Surg Hand Surg. 2014;48(1):67–73.

    Article  PubMed  Google Scholar 

  17. Kim M, Huh CH, Cho KH, Cho S. A study on the prognostic value of clinical and surgical features of dermatofibrosarcoma protuberans in Korean patients. J Eur Acad Dermatol Venereol. 2012;26(8):964–71.

    Article  CAS  PubMed  Google Scholar 

  18. Woo KJ, Bang SI, Mun GH, Oh KS, Pyon JK, Lim SY. Long-term outcomes of surgical treatment for dermatofibrosarcoma protuberans according to width of gross resection margin. J Plast Reconstr Aesthet Surg. 2016;69(3):395–401.

    Article  PubMed  Google Scholar 

  19. Kurlander DE, Martires KJ, Chen Y, Barnholtz-Sloan JS, Bordeaux JS. Risk of subsequent primary malignancies after dermatofibrosarcoma protuberans diagnosis: a national study. J Am Acad Dermatol. 2013;68(5):790–6.

    Article  PubMed  Google Scholar 

  20. Nouri K, Lodha R, Jimenez G, Robins P. Mohs micrographic surgery for dermatofibrosarcoma protuberans: University of Miami and NYU experience. Dermatol Surg. 2002;28(11):1060–4.

    PubMed  Google Scholar 

  21. Dawes KW, Hanke CW. Dermatofibrosarcoma protuberans treated with Mohs micrographic surgery: cure rates and surgical margins. Dermatol Surg. 1996;22(6):530–4.

    Article  CAS  PubMed  Google Scholar 

  22. Heuvel ST, Suurmeijer A, Pras E, Van Ginkel RJ, Hoekstra HJ. Dermatofibrosarcoma protuberans: recurrence is related to the adequacy of surgical margins. Eur J Surg Oncol. 2010;36(1):89–94.

    Article  PubMed  Google Scholar 

  23. van Houdt WJ. Margins in DFSP Reconsidered: Primum Non Nocere. Ann Surg Oncol. 2020;27(3):634–6.

    Article  PubMed  Google Scholar 

  24. O’Donnell PW, Griffin AM, Eward WC, et al. The effect of the setting of a positive surgical margin in soft tissue sarcoma. Cancer. 2014;120(18):2866–75.

    Article  PubMed  Google Scholar 

  25. England P, Hong Z, Rhea L, Hirbe A, McDonald D, Cipriano C. Does advanced imaging have a role in detecting local recurrence of soft-tissue sarcoma? Clin Orthop Relat Res. 2020;478(12):2812–20.

    Article  PubMed  PubMed Central  Google Scholar 

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Funding

No external funding was used for this study.

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Authors and Affiliations

  1. Department of Orthopedic Surgery, Mayo Clinic, Rochester, MN, USA

    Matthew T. Houdek MD, Katherine E. Mallett MD & Peter S. Rose MD

  2. Division of Orthopaedic Surgery, Department of Surgery, University of Toronto, Toronto, ON, Canada

    Kim M. Tsoi MD, PhD, Peter C. Ferguson MD, Anthony M. Griffin MSc & Jay S. Wunder MD

  3. University Musculoskeletal Oncology Unit, Mount Sinai Hospital, Toronto, ON, Canada

    Kim M. Tsoi MD, PhD, Peter C. Ferguson MD, Anthony M. Griffin MSc & Jay S. Wunder MD

  4. Mayo Clinic Alix School of Medicine, Rochester, MN, USA

    Ryan M. Claxton BS

  5. Department of Dermatology, Mayo Clinic, Rochester, MN, USA

    Christian L. Baum MD & Jerry D. Brewer MD

Authors
  1. Matthew T. Houdek MD
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  2. Kim M. Tsoi MD, PhD
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  3. Katherine E. Mallett MD
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  4. Ryan M. Claxton BS
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  5. Peter C. Ferguson MD
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  6. Anthony M. Griffin MSc
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  7. Christian L. Baum MD
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  8. Jerry D. Brewer MD
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  9. Peter S. Rose MD
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  10. Jay S. Wunder MD
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Corresponding author

Correspondence to Matthew T. Houdek MD.

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Houdek, M.T., Tsoi, K.M., Mallett, K.E. et al. Surgical Outcomes of Primary Dermatofibrosarcoma Protuberans: A Retrospective, Multicenter Study. Ann Surg Oncol 29, 8632–8638 (2022). https://doi.org/10.1245/s10434-022-12351-0

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  • DOI: https://doi.org/10.1245/s10434-022-12351-0

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