Abstract
Dermatofibrosarcoma protuberans (DFSP) is a rare, slow-growing sarcoma. DSFP has low metastatic potential but may be locally aggressive, exhibiting extensive subclinical extension and a high local recurrence rate. Treatment of disease centers on complete surgical resection with emphasis on negative histologic margins and maintaining good function and cosmesis. Treatment options include Mohs micrographic surgery (MMS), modified MMS, wide local excision (WLE), and WLE with circumferential and peripheral deep-margin pathologic assessment. MMS or modified MMS may allow lower recurrence rates than conventional WLE and may be considered first-line options. WLE with circumferential and peripheral deep-margin pathologic assessment may also enable lower recurrence rate with smaller margins. Postoperative adjuvant radiotherapy may be considered in unresectable disease. Imatinib mesylate may be used for unresectable, recurrent, and/or metastatic cases. Other targeted therapies may be beneficial. Treatment approach should take individual factors into account including patient comorbidities, tumor characteristics, and treatment availability.
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References
Kreicher KL, Kurlander DE, Gittleman HR, et al. Incidence and survival of primary dermatofibrosarcoma protuberans in the United States. Dermatol Surg. 2016;42:S24–31.
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.
Rouhani P, Fletcher CD, Devesa SS, et al. Cutaneous soft tissue sarcoma incidence patterns in the U.S.: an analysis of 12,114 cases. Cancer. 2008;113(3):616–27.
Bowne WB, Antonescu CR, Leung DH, et al. Dermatofibrosarcoma protuberans: a clinicopathologic analysis of patients treated and followed at a single institution. Cancer. 2000;88(12):2711–20.
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.
Corey RM, Swett K, Ward WG. Epidemiology and survivorship of soft tissue sarcomas in adults: a national cancer database report. Cancer Med. 2014;3(5):1404–15.
Zlatnik MG, Dinh TV, Lucci JA 3rd, et al. Dermatofibrosarcoma protuberans of the vulva: report of two new cases and review of the literature. J Low Genit Tract Dis. 1999;3(2):135–8.
Reha J, Katz SC. Dermatofibrosarcoma protuberans. Surg Clin North Am. 2016;96(5):1031–46.
Kneebone RL, Melissas J, Mannell A. Dermatofibrosarcoma protuberans in black patients. S Afr Med J. 1984;66(24):919–21.
McPeak CJ, Cruz T, Nicastri AD. Dermatofibrosarcoma protuberans: an analysis of 86 cases--five with metastasis. Ann Surg. 1967;166(5):803–16.
Ani AN, Attah EB, Ajayi OO. Dermatofibrosarcoma protruberans: analysis of eight cases in an African population. Am Surg. 1976;42(12):934–40.
Kurlander DE, Martires KJ, Chen Y, et al. Risk of subsequent primary malignancies after dermatofibrosarcoma protuberans diagnosis: a national study. J Am Acad Dermatol. 2013;68(5):790–6.
Parlette LE, Smith CK, Germain LM, et al. Accelerated growth of dermatofibrosarcoma protuberans during pregnancy. J Am Acad Dermatol. 1999;41(5 Pt 1):778–83.
Kreicher KL, Honda KS, Kurlander DE, et al. Hormone receptor expression in patients with dermatofibrosarcoma protuberans. J Am Acad Dermatol. 2016;75(6):1205–9.
Buck DW, Kim JY, Alam M, et al. Multidisciplinary approach to the management of dermatofibrosarcoma protuberans. J Am Acad Dermatol. 2012;67(5):861–6.
Miller SJ, Alam M, Andersen JS, Berg D, National Comprehensive Cancer Network, et al. Dermatofibrosarcoma protuberans. J Natl Compr Canc Netw. 2012;10(3):312–8.
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.
DuBay D, Cimmino V, Lowe L, et al. Low recurrence rate after surgery for dermatofibrosarcoma protuberans: a multidisciplinary approach from a single institution. Cancer. 2004;100(5):1008–16.
Farma JM, Ammori JB, Zager JS, et al. Dermatofibrosarcoma protuberans: how wide should we resect? Ann Surg Oncol. 2010;17(8):2112–8.
Lemm D, Mügge LO, Mentzel T, et al. Current treatment options in dermatofibrosarcoma protuberans. J Cancer Res Clin Oncol. 2009;135(5):653–65.
Massey RA, Tok J, Strippoli BA, et al. A comparison of frozen and paraffin sections in dermatofibrosarcoma protuberans. Dermatol Surg. 1998;24(9):995–8.
Bogucki B, Neuhaus I, Hurst EA. Dermatofibrosarcoma protuberans: a review of the literature. Dermatol Surg. 2012;38(4):537–51.
Foroozan M, Sei JF, Amini M, et al. Efficacy of Mohs micrographic surgery for the treatment of dermatofibrosarcoma protuberans: systematic review. Arch Dermatol. 2012;148(9):1055–63.
Criscito MC, Martires KJ, Stein JA. Prognostic factors, treatment, and survival in dermatofibrosarcoma protuberans. JAMA Dermatol. 2016;152(12):1365–71.
Castle KO, Guadagnolo BA, Tsai CJ, et al. 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.
von Mehren M, Randall RL, Benjamin RS, et al. Soft tissue sarcoma, version 2.2016, NCCN clinical practice guidelines in oncology. J Natl Compr Cancer Netw. 2016;14(6):758–86.
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.
Heuvel ST, Suurmeijer A, Pras E, et al. Dermatofibrosarcoma protuberans: recurrence is related to the adequacy of surgical margins. Eur J Surg Oncol. 2010;36(1):89–94.
Khatri VP, Galante JM, Bold RJ, et al. Dermatofibrosarcoma protuberans: reappraisal of wide local excision and impact of inadequate initial treatment. Ann Surg Oncol. 2003;10(9):1118–22.
Cai H, Wang Y, Wu J, et al. Dermatofibrosarcoma protuberans: clinical diagnoses and treatment results of 260 cases in China. J Surg Oncol. 2012;105(2):142–8.
Fields RC, Hameed M, Qin LX, et al. Dermatofibrosarcoma protuberans (DFSP): predictors of recurrence and the use of systemic therapy. Ann Surg Oncol. 2011;18(2):328–36.
Bichakjian CK, Olencki T, Alam M, et al. Dermatofibrosarcoma protuberans, version 1.2014. J Natl Compr Cancer Netw. 2014;12(6):863–8.
Erdem O, Wyatt AJ, Lin E, et al. Dermatofibrosarcoma protuberans treated with wide local excision and followed at a cancer hospital: prognostic significance of clinicopathologic variables. Am J Dermatopathol. 2012;34:24–34.
Kim BJ, Kim H, Jin US, et al. Wide local excision for dermatofibrosarcoma protuberans: a single-center series of 90 patients. Biomed Res Int. 2015;2015:642549.
Woo KJ, Bang SI, Mun GH, et al. 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.
Ratner D, Thomas CO, Johnson TM, 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.
Serra-Guillén C, Llombart B, Nagore E, et al. Mohs micrographic surgery in dermatofibrosarcoma protuberans allows tumour clearance with smaller margins and greater preservation of healthy tissue compared with conventional surgery: a study of 74 primary cases. Br J Dermatol. 2015;172(5):1303–7.
Llombart B, Serra-Guillén C, Monteagudo C, et al. Dermatofibrosarcoma protuberans: a comprehensive review and update on diagnosis and management. Semin Diagn Pathol. 2013;30(1):13–28.
Tan YG, Chia CS, Loh WL, et al. Single-institution review of managing dermatofibrosarcoma protuberans. ANZ J Surg. 2016;86(5):372–6.
Loghdey MS, Varma S, Rajpara SM, et al. Mohs micrographic surgery for dermatofibrosarcoma protuberans (DFSP): a single-centre series of 76 patients treated by frozen-section Mohs micrographic surgery with a review of the literature. J Plast Reconstr Aesthet Surg. 2014;67(10):1315–21.
Meguerditchian AN, Wang J, Lema B, et al. Wide excision or Mohs micrographic surgery for the treatment of primary dermatofibrosarcoma protuberans. Am J Clin Oncol. 2010;33(3):300–3.
Brewer JD, Roenigk RK, Otley CC. Wide local excision or Mohs micrographic surgery for primary dermatofibrosarcoma protuberans. Am J Clin Oncol. 2011;34(5):545–6.
Paradisi A, Abeni D, Rusciani A, et al. Dermatofibrosarcoma protuberans: wide local excision vs. Mohs micrographic surgery. Cancer Treat Rev. 2008;34(8):728–36.
Barlow RJ, Mallipeddi R, Sheth N. Is Mohs micrographic surgery more effective than wide local excision for treatment of dermatofibrosarcoma protuberans in reducing risk of local recurrence? A critically appraised topic. Br J Dermatol. 2013;168(1):229–30.
Matin RN, Acland KM, Williams HC. Is Mohs micrographic surgery more effective than wide local excision for treatment of dermatofibrosarcoma protuberans in reducing risk of local recurrence? A critically appraised topic. Br J Dermatol. 2012;167(1):6–9.
Riggs K, McGuigan KL, Morrison WB, et al. Role of magnetic resonance imaging in perioperative assessment of dermatofibrosarcoma protuberans. Dermatol Surg. 2009;35(12):2036–41.
Serra-Guillén C, SanmartÃn O, Llombart B, et al. Correlation between preoperative magnetic resonance imaging and surgical margins with modified Mohs for dermatofibrosarcoma protuberans. Dermatol Surg. 2011;37(11):1638–45.
Rutgers EJ, Kroon BB, Albus-Lutter CE, et al. Dermatofibrosarcoma protuberans: treatment and prognosis. Eur J Surg Oncol. 1992;18(3):241–8.
Goldblum JR, Reith JD, Weiss SW. Sarcomas arising in dermatofibrosarcoma protuberans: a reappraisal of biologic behavior in eighteen cases treated by wide local excision with extended clinical follow up. Am J Surg Pathol. 2000;24(8):1125–30.
Abbott JJ, Oliveira AM, Nascimento AG. The prognostic significance of fibrosarcomatous transformation in dermatofibrosarcoma protuberans. Am J Surg Pathol. 2006;30(4):436–43.
Liang CA, Jambusaria-Pahlajani A, Karia PS, et al. A systematic review of outcome data for dermatofibrosarcoma protuberans with and without fibrosarcomatous change. J Am Acad Dermatol. 2014;71(4):781–6.
Gloster HM Jr. Dermatofibrosarcoma protuberans. J Am Acad Dermatol. 1996;35(3 Pt 1):355–74; quiz 375–6.
Barnes L, Coleman JA Jr, Johnson JT. Dermatofibrosarcoma protuberans of the head and neck. Arch Otolaryngol. 1984;110(6):398–404.
Mark RJ, Bailet JW, Tran LM, et al. Dermatofibrosarcoma protuberans of the head and neck. A report of 16 cases. Arch Otolaryngol Head Neck Surg. 1993;119(8):891–6.
Mullen JT. Dermatofibrosarcoma protuberans: wide local excision versus Mohs micrographic surgery. Surg Oncol Clin N Am. 2016;25(4):827–39.
Tierney EP, Hanke CW. Cost effectiveness of Mohs micrographic surgery: review of the literature. J Drugs Dermatol. 2009;8(10):914–22.
Yu W, Tsoukas MM, Chapman SM, et al. Surgical treatment for dermatofibrosarcoma protuberans: the Dartmouth experience and literature review. Ann Plast Surg. 2008;60(3):288–93.
Suit H, Spiro I, Mankin HJ, et al. Radiation in management of patients with dermatofibrosarcoma protuberans. J Clin Oncol. 1996;14(8):2365–9.
Marks LB, Suit HD, Rosenberg AE, et al. Dermatofibrosarcoma protuberans treated with radiation therapy. Int J Radiat Oncol Biol Phys. 1989;17(2):379–84.
Ballo MT, Zagars GK, Pisters P, et al. The role of radiation therapy in the management of dermatofibrosarcoma protuberans. Int J Radiat Oncol Biol Phys. 1998;40(4):823–7.
Glazer ES, Prieto-Granada C, Zager JS. Current approaches to cutaneous sarcomas: dermatofibrosarcoma protuberans and cutaneous leiomyosarcoma. Curr Probl Cancer. 2015;39(4):248–57.
Haas RL, Keus RB, Loftus BM, et al. The role of radiotherapy in the local management of dermatofibrosarcoma protuberans. Soft Tissue Tumours Working Group. Eur J Cancer. 1997;33(7):1055–60.
Sun LM, Wang CJ, Huang CC, et al. Dermatofibrosarcoma protuberans: treatment results of 35 cases. Radiother Oncol. 2000;57(2):175–81.
Lindner NJ, Scarborough MT, Powell GJ, et al. Revision surgery in dermatofibrosarcoma protuberans of the trunk and extremities. Eur J Surg Oncol. 1999;25(4):392–7.
Maki RG, Awan RA, Dixon RH, et al. Differential sensitivity to imatinib of 2 patients with metastatic sarcoma arising from dermatofibrosarcoma protuberans. Int J Cancer. 2002;100(6):623–6.
Rubin BP, Schuetze SM, Eary JF, et al. Molecular targeting of platelet-derived growth factor B by imatinib mesylate in a patient with metastatic dermatofibrosarcoma protuberans. J Clin Oncol. 2002;20(17):3586–91.
Mizutani K, Tamada Y, Hara K, et al. Imatinib mesylate inhibits the growth of metastatic lung lesions in a patient with dermatofibrosarcoma protuberans. Br J Dermatol. 2004;151(1):235–7.
McArthur GA, Demetri GD, van Oosterom A, et al. Molecular and clinical analysis of locally advanced dermatofibrosarcoma protuberans treated with imatinib: Imatinib Target Exploration Consortium Study B2225. J Clin Oncol. 2005;23(4):866–73.
Rutkowski P, Debiec-Rychter M, Nowecki Z, et al. Treatment of advanced dermatofibrosarcoma protuberans with imatinib mesylate with or without surgical resection. J Eur Acad Dermatol Venereol. 2011;25:264–70.
Kerob D, Pedeutour F, Leboeuf C, et al. Value of cytogenetic analysis in the treatment of dermatofibrosarcoma protuberans. J Clin Oncol. 2008;26(10):1757–9.
Ugurel S, Mentzel T, Utikal J, et al. Neoadjuvant imatinib in advanced primary or locally recurrent dermatofibrosarcoma protuberans: a multicenter phase II DeCOG trial with long-term follow-up. Clin Cancer Res. 2014;20(2):499–510.
Kerob D, Porcher R, Ve’rola O, et al. Imatinib mesylate as a preoperative therapy in dermatofibrosarcoma: results of a multicenter phase II study on 25 patients. Clin Cancer Res. 2010;16:3288–95.
Odueyungbo M, Ratner D. Update on the use and treatment of targeted molecular inhibitors for locally advanced and metastatic non-melanoma skin cancers. Dermatol Surg. 2016;42(Suppl 1):S49–56.
Kamar FG, Kairouz VF, Sabri AN. Dermatofibrosarcoma protuberans (DFSP) successfully treated with sorafenib: case report. Clin Sarcoma Res. 2013;3(1):5.
Fu Y, Kang H, Zhao H, et al. Sunitinib for patients with locally advanced or distantly metastatic dermatofibrosarcoma protuberans but resistant to imatinib. Int J Clin Exp Med. 2015;8(5):8288–94.
Alam M, Ibrahim O, Nodzenski M, et al. Adverse events associated with Mohs micrographic surgery: multicenter prospective cohort study of 20,821 cases at 23 centers. JAMA Dermatol. 2013;149(12):1378–85.
Zbar RI. Identifying and managing those patients at risk for aborted Mohs micrographic surgery. Ann Plast Surg. 2012;68(1):67–71.
Moore J, Isler M, Barry J, et al. Major wound complication risk factors following soft tissue sarcoma resection. Eur J Surg Oncol. 2014;40(12):1671–6.
Chen YT, Tu WT, Lee WR, et al. The efficacy of adjuvant radiotherapy in dermatofibrosarcoma protuberans: a systemic review and meta-analysis. J Eur Acad Dermatol Venereol. 2016;30(7):1107–14.
Snow SN, Gordon EM, Larson PO, et al. 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.
Chang CK, Jacobs IA, Salti GI. Outcomes of surgery for dermatofibrosarcoma protuberans. Eur J Surg Oncol. 2004;30(3):341–5.
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Hau, J., Jiang, S.I.B. (2019). Dermatofibrosarcoma Protuberans. In: Alam, M. (eds) Evidence-Based Procedural Dermatology. Springer, Cham. https://doi.org/10.1007/978-3-030-02023-1_46
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