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Radiation for Dermatofibrosarcoma Protuberans

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Radiation Therapy for Sarcomas and Skin Cancers

Part of the book series: Practical Guides in Radiation Oncology ((PGRO))

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Abstract

Dermatofibrosarcoma protuberans (DFSP) is a rare, slow-growing malignancy that originates in the deep layers of the skin. It can be locally aggressive but metastasizes very rarely. DFSP is low grade. If a higher-grade fibrosarcomatous component is present, it should be treated as a soft-tissue sarcoma. Initial misdiagnosis is common, and inexperienced physicians often treat too conservatively, leading to a prolonged clinical course. Once the diagnosis is confirmed with a deep biopsy, the correct initial management is surgical excision, using a method that ensures that the margin of the specimen is completely assessed. Adjuvant radiotherapy is indicated for close/positive margins that cannot be re-resected, for unresectable disease, or for use as adjuvant therapy in a location where recurrence and second surgery would be unacceptably morbid. Imatinib has proven useful for unresectable disease unresponsive to radiation and may be helpful in attempting to convert unresectable disease to resectability.

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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  Google Scholar 

  2. Sondak VK, Cimmino VM, Lowe LM, Dubay DA, Johnson TM. Dermatofibrosarcoma protuberans: what is the best surgical approach? Surg Oncol. 1999;8(4):183–9.

    Article  Google Scholar 

  3. Snow SN, Gordon EM, Larson PO, Bagheri MM, Bentz ML, Sable DB. Dermatofibrosarcoma protuberans: a report on 29 patients treated by Mohs micrographic surgery with long-term follow-up and review of the literature. Cancer. 2004;101(1):28–38.

    Article  Google Scholar 

  4. Taylor R. Sarcomatous tumors resembling in some respects keloids. Arch Dermatol. 1890;8:384–7.

    Google Scholar 

  5. Llombart B, Serra-Guillen C, Monteagudo C, Lopez Guerrero JA, Sanmartin O. Dermatofibrosarcoma protuberans: a comprehensive review and update on diagnosis and management. Semin Diagn Pathol. 2013;30(1):13–28.

    Article  Google Scholar 

  6. McPeak CJ, Cruz T, Nicastri AD. Dermatofibrosarcoma protuberans: an analysis of 86 cases—five with metastasis. Ann Surg 1967;166(5):803–816.

    Google Scholar 

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

    Article  Google Scholar 

  8. 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  Google Scholar 

  9. Chang CK, Jacobs IA, Salti GI. Outcomes of surgery for dermatofibrosarcoma protuberans. Eur J Surg Oncol. 2004;30(3):341–5.

    Article  Google Scholar 

  10. 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  Google Scholar 

  11. American Joint Committee on Cancer. AJCC cancer staging manual. 7th ed. New York, NY: Springer; 2010.

    Book  Google Scholar 

  12. American Joint Committee on Cancer. AJCC cancer staging manual. 8th ed. New York, NY: Springer; 2018.

    Google Scholar 

  13. Page EH, Assaad DM. Atrophic dermatofibroma and dermatofibrosarcoma protuberans. J Am Acad Dermatol. 1987;17(6):947–50.

    Article  Google Scholar 

  14. Green JJ, Heymann WR. Dermatofibrosarcoma protuberans occurring in a smallpox vaccination scar. J Am Acad Dermatol. 2003;48(5 Suppl):S54–5.

    Article  Google Scholar 

  15. Tanaka A, Hatoko M, Tada H, Kuwahara M, Iioka H, Niitsuma K. Dermatofibrosarcoma protuberans arising from a burn scar of the axilla. Ann Plast Surg. 2004;52(4):423–5.

    Article  Google Scholar 

  16. Lloyd M, Lyons I, Deodhar A, Flemming AS. Dermatofibrosarcoma protuberans arising in a bacillus Calmette-Guerin vaccination scar. Eur J Plast Surg. 2009;32(1):37–8.

    Article  Google Scholar 

  17. National Comprehensive Cancer Network. Dermatofibrosarcoma Protuberans (Version 1.2020) 2019 [Available from: https://www.nccn.org/professionals/physician_gls/PDF/dfsp.pdf.

  18. Ogawa R, Akaishi S, Hyakusoku H. Differential and exclusive diagnosis of diseases that resemble keloids and hypertrophic scars. Ann Plast Surg. 2009;62(6):660–4.

    Article  Google Scholar 

  19. Pisters PWT, O’Sullivan B. Clinical presentation, classification, and diagnosis. In: Kufe DW, Pollock RE, Weichselbaum RR, editors. Holland-Frei cancer medicine. 6th ed. Hamilton, ON: BC Decker; 2003.

    Google Scholar 

  20. Noujaim J, Thway K, Fisher C, Jones RL. Dermatofibrosarcoma protuberans: from translocation to targeted therapy. Cancer Biol Med. 2015;12(4):375–84.

    Google Scholar 

  21. Llombart B, Serra C, Requena C, Alsina M, Morgado-Carrasco D, Traves V, et al. Guidelines for diagnosis and treatment of cutaneous sarcomas: dermatofibrosarcoma protuberans. Actas Dermosifiliogr. 2018;109(10):868–77.

    Article  Google Scholar 

  22. Stacchiotti S, Pantaleo MA, Negri T, Astolfi A, Tazzari M, Dagrada GP, et al. Efficacy and biological activity of imatinib in metastatic dermatofibrosarcoma protuberans (DFSP). Clin Cancer Res. 2016;22(4):837–46.

    Article  Google Scholar 

  23. Mendenhall WM, Zlotecki RA, Scarborough MT. Dermatofibrosarcoma protuberans. Cancer. 2004;101(11):2503–8.

    Article  Google Scholar 

  24. Goldblum JR. CD34 positivity in fibrosarcomas which arise in dermatofibrosarcoma protuberans. Arch Pathol Lab Med. 1995;119(3):238–41.

    Google Scholar 

  25. Hayakawa K, Matsumoto S, Ae K, Tanizawa T, Gokita T, Funauchi Y, et al. Risk factors for distant metastasis of dermatofibrosarcoma protuberans. J Orthop Traumatol. 2016;17(3):261–6.

    Article  Google Scholar 

  26. 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  Google Scholar 

  27. Ballo MT, Zagars GK, Pisters P, Pollack A. The role of radiation therapy in the management of dermatofibrosarcoma protuberans. Int J Radiat Oncol Biol Phys. 1998;40(4):823–7.

    Article  Google Scholar 

  28. Riggs K, McGuigan KL, Morrison WB, Samie FH, Humphreys T. Role of magnetic resonance imaging in perioperative assessment of dermatofibrosarcoma protuberans. Dermatol Surg. 2009;35(12):2036–41.

    Article  Google Scholar 

  29. Ratner D, Thomas CO, Johnson TM, Sondak VK, Hamilton TA, Nelson BR, et al. Mohs micrographic surgery for the treatment of dermatofibrosarcoma protuberans. Results of a multiinstitutional series with an analysis of the extent of microscopic spread. J Am Acad Dermatol. 1997;37(4):600–13.

    Article  Google Scholar 

  30. Fiore M, Miceli R, Mussi C, Lo Vullo S, Mariani L, Lozza L, et al. Dermatofibrosarcoma protuberans treated at a single institution: a surgical disease with a high cure rate. J Clin Oncol. 2005;23(30):7669–75.

    Article  Google Scholar 

  31. Zager JS, Bui MM, Farma JM, Messina JL, Sondak VK. Proper margins of excision in dermatofibrosarcoma protuberans: wide or narrow? Ann Surg Oncol. 2008;15(9):2614–6.

    Article  Google Scholar 

  32. Kimmel Z, Ratner D, Kim JY, Wayne JD, Rademaker AW, Alam M. Peripheral excision margins for dermatofibrosarcoma protuberans: a meta-analysis of spatial data. Ann Surg Oncol. 2007;14(7):2113–20.

    Article  Google Scholar 

  33. Bowne WB, Antonescu CR, Leung DH, Katz SC, Hawkins WG, Woodruff JM, et al. Dermatofibrosarcoma protuberans: a clinicopathologic analysis of patients treated and followed at a single institution. Cancer. 2000;88(12):2711–20.

    Article  Google Scholar 

  34. Liu MZ, Wang XS, Cai L, Liu H, Chen EC, Cui NJ. Prognostic factors and treatment of 74 patients with dermatofibro-sarcoma protuberans. Zhonghua Zhong Liu Za Zhi. 2005;27(2):122–5.

    Google Scholar 

  35. Cai H, Wang Y, Wu J, Shi Y. Dermatofibrosarcoma protuberans: clinical diagnoses and treatment results of 260 cases in China. J Surg Oncol. 2012;105(2):142–8.

    Article  Google Scholar 

  36. 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.

    Google Scholar 

  37. Hobbs ER, Wheeland RG, Bailin PL, Ratz JL, Yetman RJ, Zins JE. Treatment of dermatofibrosarcoma protuberans with Mohs micrographic surgery. Ann Surg. 1988;207(1):102–7.

    Article  Google Scholar 

  38. Robinson JK. Dermatofibrosarcoma protuberans resected by Mohs' surgery (chemosurgery). A 5-year prospective study. J Am Acad Dermatol. 1985;12(6):1093–8.

    Article  Google Scholar 

  39. DuBay D, Cimmino V, Lowe L, Johnson TM, Sondak VK. Low recurrence rate after surgery for dermatofibrosarcoma protuberans: a multidisciplinary approach from a single institution. Cancer. 2004;100(5):1008–16.

    Article  Google Scholar 

  40. 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  Google Scholar 

  41. 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  Google Scholar 

  42. Loss L, Zeitouni NC. Management of scalp dermatofibrosarcoma protuberans. Dermatol Surg. 2005;31(11 Pt 1):1428–33.

    Google Scholar 

  43. Chen YT, Tu WT, Lee WR, Huang YC. The efficacy of adjuvant radiotherapy in dermatofibrosarcoma protuberans: a systemic review and meta-analysis. J Eur Acad Dermatol Venereol. 2016;30(7):1107–14.

    Article  Google Scholar 

  44. Suit H, Spiro I, Mankin HJ, Efird J, Rosenberg AE. Radiation in management of patients with dermatofibrosarcoma protuberans. J Clin Oncol. 1996;14(8):2365–9.

    Article  Google Scholar 

  45. Sun LM, Wang CJ, Huang CC, Leung SW, Chen HC, Fang FM, et al. Dermatofibrosarcoma protuberans: treatment results of 35 cases. Radiother Oncol. 2000;57(2):175–81.

    Article  Google Scholar 

  46. Haas RL, Keus RB, Loftus BM, Rutgers EJ, van Coevorden F, Bartelink H. The role of radiotherapy in the local management of dermatofibrosarcoma protuberans. Soft Tissue Tumours Working Group. Eur J Cancer. 1997;33(7):1055–60.

    Article  Google Scholar 

  47. Wang X, Liu M, Liu H, Cui N. The role of radiotherapy in 74 patients with dermatofibrosarcoma protuberans. Chin-Ger J Clin Oncol. 2006;5(6):454–7.

    Article  Google Scholar 

  48. 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.

    Article  Google Scholar 

  49. Rutkowski P, Van Glabbeke M, Rankin CJ, Ruka W, Rubin BP, Debiec-Rychter M, et al. Imatinib mesylate in advanced dermatofibrosarcoma protuberans: pooled analysis of two phase II clinical trials. J Clin Oncol. 2010;28(10):1772–9.

    Article  Google Scholar 

  50. Rutkowski P, Debiec-Rychter M, Nowecki Z, Michej W, Symonides M, Ptaszynski K, et al. Treatment of advanced dermatofibrosarcoma protuberans with imatinib mesylate with or without surgical resection. J Eur Acad Dermatol Venereol. 2011;25(3):264–70.

    Article  Google Scholar 

  51. Wicherts DA, van Coevorden F, Klomp HM, van Huizum MA, Kerst JM, Haas RL, et al. Complete resection of recurrent and initially unresectable dermatofibrosarcoma protuberans downsized by Imatinib. World J Surg Oncol. 2013;11:59.

    Article  Google Scholar 

  52. Hong JY, Liu X, Mao M, Li M, Choi DI, Kang SW, et al. Genetic aberrations in imatinib-resistant dermatofibrosarcoma protuberans revealed by whole genome sequencing. PLoS One. 2013;8(7):e69752.

    Article  ADS  Google Scholar 

  53. Marks LB, Suit HD, Rosenberg AE, Wood WC. Dermatofibrosarcoma protuberans treated with radiation therapy. Int J Radiat Oncol Biol Phys. 1989;17(2):379–84.

    Article  Google Scholar 

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Authors and Affiliations

  1. Department of Radiation Oncology, University of Florida College of Medicine, Gainesville, FL, USA

    Kathryn E. Hitchcock & William M. Mendenhall

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  1. Kathryn E. Hitchcock
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  2. William M. Mendenhall
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Correspondence to Kathryn E. Hitchcock .

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Editors and Affiliations

  1. Department of Radiation Oncology, University of Washington, Seattle, WA, USA

    Edward Kim

  2. Department of Radiation Oncology, University of Washington, Seattle, WA, USA

    Upendra Parvathaneni

  3. Department of Radiation Oncology, The Ohio State University James Cancer Center, Columbus, OH, USA

    Meng Xu Welliver

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Hitchcock, K.E., Mendenhall, W.M. (2022). Radiation for Dermatofibrosarcoma Protuberans. In: Kim, E., Parvathaneni, U., Welliver, M.X. (eds) Radiation Therapy for Sarcomas and Skin Cancers. Practical Guides in Radiation Oncology. Springer, Cham. https://doi.org/10.1007/978-3-031-06706-8_11

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  • DOI: https://doi.org/10.1007/978-3-031-06706-8_11

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  • Publisher Name: Springer, Cham

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  • Online ISBN: 978-3-031-06706-8

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