Overview
Dermatofibrosarcoma protuberans (DFSP) is an uncommon, low-grade sarcoma of fibroblast origin with an incidence rate of 4.1 to 4.5 cases per million persons per year in the United States.1–4 A predilection for occurring in African Americans has been reported in one study.3 Initial misdiagnosis, prolonged time to accurate diagnosis, and large tumor size at the time of diagnosis are common. However, DFSP rarely metastasizes.5 When metastasis occurs, it is typically in the lung, bone, or regional lymph nodes. Three-dimensional reconstruction of DFSP6 has revealed tumors with highly irregular shapes and frequent finger-like extensions.7 As a result, incomplete removal and subsequent recurrence are common without attention to full assessment of the peripheral and deep margin. The local recurrence rate for wide local excision (WLE) of DFSP in studies ranges from 10% to 60%, whereas the rate of development of regional or distant metastatic disease is only 1% and 4%–7.4%, respectively.8,9
Guidelines Update Methodology
The complete details of the Development and Update of the NCCN Guidelines are available at NCCN.org.
Literature Search Criteria and Guidelines Update Methodology
Prior to the update of this version of the NCCN Clinical Practice Guidelines in Oncology (NCCN Guidelines) for Dermatofibrosarcoma Protuberans, an electronic search of the PubMed database was performed to obtain key literature using the following search term: dermatofibrosarcoma protuberans. The PubMed database was chosen as it remains the most widely used resource for medical literature and indexes peer-reviewed biomedical literature.10
The search results were narrowed by selecting studies in humans published in English. Results were confined to the following article types: “clinical trial”; “guideline”; “meta-analysis”; “practice guideline”; “randomized controlled trial”; “systematic reviews”; and “validation studies.”
The data from key PubMed articles as well as articles from additional sources deemed as relevant to these guidelines as discussed by the panel during the NCCN Guidelines update have been included in this version of the discussion section. Recommendations for which high-level evidence is lacking are based on the panel’s review of lower-level evidence and expert opinion.
Sensitive/Inclusive Language
NCCN Guidelines strive to use language that advances the goals of equity, inclusion, and representation.11 NCCN Guidelines endeavor to use language that is person-first; not stigmatizing; antiracist, anticlassist, antimisogynist, antiageist, antiableist, and anti–weight biased; and inclusive of individuals of all sexual orientations and gender identities. NCCN Guidelines incorporate nongendered language, instead focusing on organ-specific recommendations. This language is both more accurate and more inclusive and can help fully address the needs of individuals of all sexual orientations and gender identities. NCCN Guidelines will continue to use the terms “men,” “women,” “female,” and “male” when citing statistics, recommendations, or data from organizations or sources that do not use inclusive terms. Most studies do not report how sex and gender data are collected and use these terms interchangeably or inconsistently. If sources do not differentiate gender from sex assigned at birth or organs present, the information is presumed to predominantly represent cisgender individuals. NCCN encourages researchers to collect more specific data in future studies and organizations to use more inclusive and accurate language in their future analyses.
Evaluation
Histologically, DFSP typically presents as a storiform or fascicular proliferation of bland spindled cells that extends from the dermis into the subcutis.12,13 Virtually all cases are CD34-positive and factor XIIIa-negative with rare exceptions.14,15 Currently, no synoptic reporting is recommended. Preliminary workup for DFSP consists of history and physical examination and biopsy (Figure 1). It should be noted that this tumor is frequently misdiagnosed due to inadequate tissue sampling resulting from shallow biopsy. As the superficial aspect of a DFSP may not be distinct from benign lesions, a punch or incisional biopsy that samples the subcutaneous layer is strongly recommended. If a biopsy is indeterminate or clinical suspicion remains, rebiopsy is recommended.
DFSP-1. NCCN Clinical Practice Guidelines in Oncology (NCCN Guidelines) for Dermatofibrosarcoma Protuberans, Version 1.2025.
Citation: Journal of the National Comprehensive Cancer Network 23, 1; 10.6004/jnccn.2025.0001
In most cases, examination of hematoxylin and eosin–stained specimens by light microscopy results in an unequivocal diagnosis. However, differentiation of DFSP from dermatofibroma can be difficult at times. Staining with CD34,15,16 factor XIIIa,14,17 and other immunomarkers such as stromelysins 3, nestin, apolipoprotein D, and cathepsin K,18–21 might be useful in such instances. The NCCN panel recommends that appropriate and confirmatory immunostaining be performed in all cases of suspected DFSP.
Whether the histologic features of a high mitotic rate or evidence of fibrosarcomatous (FS-DFSP) change have prognostic significance in DFSP is unclear.22,23 Studies in the biomedical literature both support24–32 the connection between FS-DFSP and an increased risk of local recurrence, lower time to recurrence, and increased risk of metastasis, and refute33,34 this notion. A systematic review of 225 patients with FS-DFSP and 1,422 with DFSP reported risks of local recurrence (29.8% vs 13.7%), metastasis (14.4% vs 1.1%), and death (14.7% vs 0.8%) from the disease to be significantly higher in FS-DFSP.35 Overall, FS-DFSP is associated with a metastatic risk range of 10%–23.5%.23,26,36 The NCCN panel recommends that the debulking specimens from all excisions should be examined to identify FS transformation of DFSP. If FS transformation or other sarcoma subtypes are found, multidisciplinary consultation for consideration of further treatment and surveillance is recommended. Clinicians should consult the NCCN Guidelines for Soft Tissue Sarcoma (available at NCCN.org) for multimodal therapy and surveillance considerations including CT of draining nodal basin and chest.
After DFSP confirmation, additional workup may include a complete skin examination and consideration of preoperative MRI with contrast for treatment planning if there is suspicion of extensive subcutaneous extension. Because decisions about diagnosis and resection may be complex, multidisciplinary consultation at a center with specialized expertise should be strongly considered, especially for large or recurrent DFSP because it may optimize clinical and reconstructive outcomes.37,38
Treatment
Initial treatment of DFSP is surgical (Figure 2). Because of its proclivity for irregular and frequently deep subclinical extensions, every effort should be made to completely remove this tumor at initial therapy. Excision with Mohs micrographic surgery (Mohs) or other forms of peripheral and deep en face margin assessment (PDEMA) is recommended over WLE. En face sectioning is preferred to prevent missing small foci of tumor. The most commonly used form of PDEMA is Mohs (See NCCN Guidelines for Squamous Cell Skin Cancer – Principles of PDEMA Technique, available at NCCN.org).39 When anatomic structures at the deep margin (eg, major vessels, nerves, bone) preclude complete histologic evaluation of the marginal surface via Mohs or other forms of PDEMA, these surgical techniques should be used to evaluate as much of the marginal surface as feasible. A combination of PDEMA and WLE for the deep margin has been reported in the literature.38 Treatment considerations for nonvisualized areas may be the subject of multidisciplinary discussion. If PDEMA is unavailable, WLE can be considered. Wide undermining is discouraged prior to confirmation of clear margins due to the difficulty of interpreting subsequent re-excised margins, and the risk of concealing residual tumor below mobilized tissue. Additionally, tumor mutation analysis and neoadjuvant imatinib can be considered options for unresectable or borderline resectable disease. Consider neoadjuvant imatinib for patients in whom DFSP resection with negative margins may result in unacceptable functional or cosmetic outcomes.40 The NCCN panel recommends that if a negative margin is achieved, no adjuvant treatment is necessary. When Mohs or other forms of PDEMA are used, radiation therapy is not recommended.
DFSP-2. NCCN Clinical Practice Guidelines in Oncology (NCCN Guidelines) for Dermatofibrosarcoma Protuberans, Version 1.2025.
Citation: Journal of the National Comprehensive Cancer Network 23, 1; 10.6004/jnccn.2025.0001
If initial surgery yields positive margins, reresection is recommended whenever possible, with the goal of achieving clear margins (Figure 3). Mohs or modified Mohs surgery, and traditional WLE with wider margins, which has been associated with higher tumor clearance and lower rates of recurrence,41–43 are all methods to achieve complete histologic assessment. Studies examining outcomes of both Mohs and WLE have consistently reported lower recurrence rates for the former (0%–6.6% vs 1.7%–30.8%).44–53A large retrospective series of 204 patients with DFSP showed a very low local recurrence rate (1%) using WLE with total peripheral margin pathologic evaluation, underscoring the importance of meticulous pathologic margin evaluation with any surgical technique.54 This notion was also supported by more studies.55,56 It is recommended that any reconstruction involving extensive undermining be avoided. Tissue rearrangement, if necessary, should be delayed until negative histologic margins are verified to prevent displacing a potentially positive margin or hampering interpretation of re-excisions. If there is concern that the surgical margins are not clear when Mohs or PDEMA is not available, split-thickness skin grafting should be considered to monitor for recurrence.
DFSP-B. NCCN Clinical Practice Guidelines in Oncology (NCCN Guidelines) for Dermatofibrosarcoma Protuberans, Version 1.2025.
Citation: Journal of the National Comprehensive Cancer Network 23, 1; 10.6004/jnccn.2025.0001
Radiation has occasionally been used as a primary therapeutic modality for DFSP along with other therapies,57–59 but it is most beneficial as adjuvant therapy after surgery.57–64 In a single-institution retrospective review of 53 patients with DFSP treated with surgery and preoperative or postoperative radiation therapy, local control was 93% and actuarial overall survival was 98% at 10 years.34 Another small patient series reported that 86% of patients with DFSP treated with postoperative radiation therapy remained disease-free at a median follow-up of 10.5 years.63 In a systematic review and meta-analysis of adjuvant radiation therapy for DFSP after WLE, the overall recurrence rate was reported to be 11.74%. Patients with positive/close margins had a recurrence rate of 14.23%, whereas those with negative margins had no recurrence.65 The NCCN panel recommends that when Mohs or other forms of PDEMA are not used, radiation therapy can be considered if margins are deemed narrow by the treating physicians. Radiation therapy can be considered for the treatment of positive margins if not given previously and further resection is not feasible (Figure 4).
DFSP-C. NCCN Clinical Practice Guidelines in Oncology (NCCN Guidelines) for Dermatofibrosarcoma Protuberans, Version 1.2025.
Citation: Journal of the National Comprehensive Cancer Network 23, 1; 10.6004/jnccn.2025.0001
DFSP can be treated by targeted platelet-derived growth factor receptors (Figure 5). DFSP is characterized by a translocation between chromosomes 17 and 22 [t(17;22)(q22;q13)] resulting in the overexpression of platelet-derived growth factor receptor β.66–68 These findings suggest that targeting platelet-derived growth factor receptors may be an effective treatment of DFSP. In published results, imatinib mesylate, a protein tyrosine kinase inhibitor, has shown clinical activity against DFSP,40,69–73 which has led to its approval by the US FDA for the treatment of unresectable, recurrent, and/or metastatic DFSP in adult patients. It is still unclear whether and the extent to which the COL1A1-PDGFB fusion gene dictates imatinib response.70 In a systematic review that included patients receiving imatinib as monotherapy, adjuvant, or neoadjuvant therapy, complete response, partial response, stable disease, and progressive disease were reported in 5.2%, 55.2%, 27.6%, and 9.2% of patients, respectively.70 In the neoadjuvant setting, complete response, partial response, stable disease, and progressive disease rates were reported to be 7.1%, 50%, 35.7%, and 7.1%, respectively.40
DFSP-A. NCCN Clinical Practice Guidelines in Oncology (NCCN Guidelines) for Dermatofibrosarcoma Protuberans, Version 1.2025.
Citation: Journal of the National Comprehensive Cancer Network 23, 1; 10.6004/jnccn.2025.0001
Follow-up
Given the historically high local recurrence rates for DFSP, ongoing clinical follow-up with focus on the primary site every 6 to 12 months is indicated, with rebiopsy of any suspicious regions. Although metastatic disease is rare, a guided history and physical and patient education about regular self-examination are recommended. Consider MRI surveillance for deeply invasive disease or other concerns related to recurrence.
Recurrent tumors should be resected whenever possible (Figure 6). Adjuvant radiation therapy may be considered after surgery. For patients who are not surgical candidates, radiation therapy alone is an option if not given previously. Imatinib mesylate should be considered in cases where the disease is unresectable after multiple resections, or if unacceptable functional or cosmetic outcomes will occur with further resection. It is recommended that the tumor mutation is confirmed with fluorescence in-situ hybridization for PDGRF translocation.
DFSP-3. NCCN Clinical Practice Guidelines in Oncology (NCCN Guidelines) for Dermatofibrosarcoma Protuberans, Version 1.2025.
Citation: Journal of the National Comprehensive Cancer Network 23, 1; 10.6004/jnccn.2025.0001
In the rare event of metastatic disease, multidisciplinary consultation is recommended to coordinate treatment (see the NCCN Guidelines for Soft Tissue Sarcoma, available at NCCN.org).
References
- 1.↑
Criscione VD, Weinstock MA. Descriptive epidemiology of dermatofibrosarcoma protuberans in the United States, 1973 to 2002. J Am Acad Dermatol 2007;56:968–973.
- 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;113:616–627.
- 3.↑
Kreicher KL, Kurlander DE, Gittleman HR, et al. Incidence and Survival of Primary Dermatofibrosarcoma Protuberans in the United States. Dermatol Surg 2016;42(Suppl 1):S24–31.
- 4.↑
Criscito MC, Martires KJ, Stein JA. Prognostic factors, treatment, and survival in dermatofibrosarcoma protuberans. JAMA Dermatol 2016;152:1365–1371.
- 5.↑
Martin L, Piette F, Blanc P, et al. Clinical variants of the preprotuberant stage of dermatofibrosarcoma protuberans. Br J Dermatol 2005;153:932–936.
- 6.↑
Haycox CL, Odland PB, Olbricht SM, Casey B. Dermatofibrosarcoma protuberans (DFSP): growth characteristics based on tumor modeling and a review of cases treated with Mohs micrographic surgery. Ann Plast Surg 1997;38:246–251.
- 7.↑
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;37:600–613.
- 8.↑
Hayakawa K, Matsumoto S, Ae K, et al. Risk factors for distant metastasis of dermatofibrosarcoma protuberans. J Orthop Traumatol 2016;17:261–266.
- 9.↑
Vidimos AT, Helm TN, Papay FA. Dermatofibrosarcoma protuberans. In: Cutaneous Oncology: Pathophysiology, Diagnosis, and Management. Malden, MA: Blackwell Scientific; 1998.
- 10.↑
National Library of Medicine. PubMed Overview. Accessed November 21, 2024. Available at: https://pubmed.ncbi.nlm.nih.gov/about/
- 11.↑
Freedman-Cass DA, Fischer T, Alpert AB, et al. The value and process of inclusion: using sensitive, respectful, and inclusive language and images in NCCN content. J Natl Compr Canc Netw 2023;21:434–441.
- 12.↑
Mentzel T, Pedeutour F, Lazar A, Coindre JM. Dermatofibrosarcoma Protuberans. In: Fletcher CD, Bridge JA, Hogendoorn PCW, Mertens F, eds. WHO Classification of Tumors: Soft Tissue and Bone, 4th ed. Lyon, France: IARC Press; 2013:77–79.
- 13.↑
Calonje E, Brenn T, Lazar A, McKee PH. Connective Tissue Tumors. In: Calonje E, Brenn T, Lazar A, McKee PH, eds. McKee’s Pathology of the Skin with Clinical Correlations, 4th ed. Philadelphia, PA: Elsevier Saunders; 2012:1630–1635.
- 14.↑
Abenoza P, Lillemoe T. CD34 and factor XIIIa in the differential diagnosis of dermatofibroma and dermatofibrosarcoma protuberans. Am J Dermatopathol 1993;15:429–434.
- 15.↑
Aiba S, Tabata N, Ishii H, et al. Dermatofibrosarcoma protuberans is a unique fibrohistiocytic tumour expressing CD34. Br J Dermatol 1992;127:79–84.
- 16.↑
Kutzner H. Expression of the human progenitor cell antigen CD34 (HPCA-1) distinguishes dermatofibrosarcoma protuberans from fibrous histiocytoma in formalin-fixed, paraffin-embedded tissue. J Am Acad Dermatol 1993;28:613–617.
- 17.↑
Goldblum JR, Tuthill RJ. CD34 and factor-XIIIa immunoreactivity in dermatofibrosarcoma protuberans and dermatofibroma. Am J Dermatopathol 1997;19:147–153.
- 18.↑
Sellheyer K, Nelson P, Krahl D. Dermatofibrosarcoma protuberans: a tumour of nestin-positive cutaneous mesenchymal stem cells? Br J Dermatol 2009;161:1317–1322.
- 19.↑
Cribier B, Noacco G, Peltre B, Grosshans E. Stromelysin 3 expression: a useful marker for the differential diagnosis dermatofibroma versus dermatofibrosarcoma protuberans. J Am Acad Dermatol 2002;46:408–413.
- 20.↑
Lisovsky M, Hoang MP, Dresser KA, et al. Apolipoprotein D in CD34- positive and CD34-negative cutaneous neoplasms: a useful marker in differentiating superficial acral fibromyxoma from dermatofibrosarcoma protuberans. Mod Pathol 2008;21:31–38.
- 21.↑
Yan X, Takahara M, Xie L, et al. Cathepsin K expression: a useful marker for the differential diagnosis of dermatofibroma and dermatofibrosarcoma protuberans. Histopathology 2010;57:486–488.
- 23.↑
Szollosi Z, Nemes Z. Transformed dermatofibrosarcoma protuberans: a clinicopathological study of eight cases. J Clin Pathol 2005;58:751–756.
- 24.↑
Mentzel T, Beham A, Katenkamp D, et al. Fibrosarcomatous (“high-grade") dermatofibrosarcoma protuberans: clinicopathologic and immunohistochemical study of a series of 41 cases with emphasis on prognostic significance. Am J Surg Pathol 1998;22:576–587.
- 25.↑
Bowne WB, Antonescu CR, Leung DH, et al. Dermatofibrosarcoma protuberans: a clinicopathologic analysis of patients treated and followed at a single institution. Cancer 2000;88:2711–2720.
- 26.↑
Abbott JJ, Oliveira AM, Nascimento AG. The prognostic significance of fibrosarcomatous transformation in dermatofibrosarcoma protuberans. Am J Surg Pathol 2006;30:436–443.
- 27.↑
Ding J, Hashimoto H, Enjoji M. Dermatofibrosarcoma protuberans with fibrosarcomatous areas. A clinicopathologic study of nine cases and a comparison with allied tumors. Cancer 1989;64:721–729.
- 28.↑
Hoesly PM, Lowe GC, Lohse CM, et al. Prognostic impact of fibrosarcomatous transformation in dermatofibrosarcoma protuberans: a cohort study. J Am Acad Dermatol 2015;72:419–425.
- 29.↑
Lyu A, Wang Q. Dermatofibrosarcoma protuberans: a clinical analysis. Oncol Lett 2018;16:1855–1862.
- 30.↑
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.
- 31.↑
Jing C, Zhang H, Zhang X, Yu S. Dermatofibrosarcoma protuberans: a clinicopathologic and therapeutic analysis of 254 cases at a single institution. Dermatol Surg 2021;47:e26–30.
- 32.↑
Li Y, Wang C, Xiang B, et al. Clinical features, pathological findings and treatment of recurrent dermatofibrosarcoma protuberans. J Cancer 2017;8:1319–1323.
- 33.↑
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;24:1125–1130.
- 34.↑
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;86:585–590.
- 35.↑
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;71:781–786.
- 36.↑
Cai H, Wang Y, Wu J, Shi Y. Dermatofibrosarcoma protuberans: clinical diagnoses and treatment results of 260 cases in China. J Surg Oncol 2012;105:142–148.
- 37.↑
Buck DW, 2nd, Kim JY, Alam M, et al. Multidisciplinary approach to the management of dermatofibrosarcoma protuberans. J Am Acad Dermatol 2012;67:861–866.
- 38.↑
Chappell AG, Doe SC, Worley B, et al. Multidisciplinary surgical treatment approach for dermatofibrosarcoma protuberans: an update. Arch Dermatol Res 2021;313:367–372.
- 39.↑
Curtis KK, Fakult NJ, Strunck JL, et al. Establishing consensus for Mohs micrographic surgical techniques in the treatment of melanoma in situ for future clinical trials: a modified Delphi study. J Natl Compr Canc Netw 2024;22:1–6.
- 40.↑
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;20:499–510.
- 41.↑
Zhou X, Sun D, Liu Y, et al. Dermatofibrosarcoma protuberans: our 10-year experience on 80 patients. J Dermatolog Treat 2020;31:554–558.
- 42.↑
Kimmel Z, Ratner D, Kim JY, et al. Peripheral excision margins for dermatofibrosarcoma protuberans: a meta-analysis of spatial data. Ann Surg Oncol 2007;14:2113–2120.
- 43.↑
Chen Y, Jiang G. Association between surgical excision margins and outcomes in patients with dermatofibrosarcoma protuberans: a meta-analysis. Dermatol Ther 2021;34:e14954.
- 44.↑
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;35:82–87.
- 45.↑
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;100:1008–1016.
- 46.↑
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;33:300–303.
- 47.↑
Foroozan M, Sei JF, Amini M, et al. Efficacy of Mohs micrographic surgery for the treatment of dermatofibrosarcoma protuberans: systematic review. Arch Dermatol 2012;148:1055–1063.
- 48.↑
Bogucki B, Neuhaus I, Hurst EA. Dermatofibrosarcoma protuberans: a review of the literature. Dermatol Surg 2012;38:537–551.
- 49.↑
Paradisi A, Abeni D, Rusciani A, et al. Dermatofibrosarcoma protuberans: wide local excision vs. Mohs micrographic surgery. Cancer Treat Rev 2008;34:728–736.
- 50.↑
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;43:98–106.
- 51.↑
Veronese F, Boggio P, Tiberio R, et al. Wide local excision vs. Mohs Tübingen technique in the treatment of dermatofibrosarcoma protuberans: a two-centre retrospective study and literature review. J Eur Acad Dermatol Venereol 2017;31:2069–2076.
- 52.↑
Malan M, Xuejingzi W, Quan SJ. The efficacy of Mohs micrographic surgery over the traditional wide local excision surgery in the cure of dermatofibrosarcoma protuberans. Pan Afr Med J 2019;33:297.
- 53.↑
Durack A, Gran S, Gardiner MD, et al. A 10-year review of surgical management of dermatofibrosarcoma protuberans. Br J Dermatol 2021;184:731–739.
- 54.↑
Farma JM, Ammori JB, Zager JS, et al. Dermatofibrosarcoma protuberans: how wide should we resect? Ann Surg Oncol 2010;17:2112–2118.
- 55.↑
Snow H, Davies E, Strauss DC, et al. Conservative re-excision is a safe and simple alternative to radical resection in revision surgery for dermatofibrosarcoma protuberans. Ann Surg Oncol 2020;27:919–923.
- 56.↑
Harati K, Lange K, Goertz O, et al. A single-institutional review of 68 patients with dermatofibrosarcoma protuberans: wide re-excision after inadequate previous surgery results in a high rate of local control. World J Surg Oncol 2017;15:5.
- 57.↑
Suit H, Spiro I, Mankin HJ, et al. Radiation in management of patients with dermatofibrosarcoma protuberans. J Clin Oncol 1996;14:2365–2369.
- 58.↑
Uysal B, Sager O, Gamsiz H, et al. Evaluation of the role of radiotherapy in the management of dermatofibrosarcoma protuberans. J BUON 2013;18:268–273.
- 59.↑
Hamid R, Hafeez A, Darzi MA, et al. Outcome of wide local excision in dermatofibrosarcoma protuberans and use of radiotherapy for margin-positive disease. Indian Dermatol Online J 2013;4:93–96.
- 60.↑
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;40:823–827.
- 61.↑
Dagan R, Morris CG, Zlotecki RA, et al. Radiotherapy in the treatment of dermatofibrosarcoma protuberans. Am J Clin Oncol 2005;28:537–539.
- 62.↑
Sun LM, Wang CJ, Huang CC, et al. Dermatofibrosarcoma protuberans: treatment results of 35 cases. Radiother Oncol 2000;57:175–181.
- 63.↑
Williams N, Morris CG, Kirwan JM, et al. Radiotherapy for dermatofibrosarcoma protuberans. Am J Clin Oncol 2014;37:430–432.
- 64.↑
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;33:1055–1060.
- 65.↑
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;30:1107–1114.
- 66.↑
Takahira T, Oda Y, Tamiya S, et al. Detection of COL1A1-PDGFB fusion transcripts and PDGFB/PDGFRB mRNA expression in dermatofibrosarcoma protuberans. Mod Pathol 2007;20:668–675.
- 67.↑
Patel KU, Szabo SS, Hernandez VS, et al. Dermatofibrosarcoma protuberans COL1A1-PDGFB fusion is identified in virtually all dermatofibrosarcoma protuberans cases when investigated by newly developed multiplex reverse transcription polymerase chain reaction and fluorescence in situ hybridization assays. Hum Pathol 2008;39:184–193.
- 68.↑
Simon MP, Pedeutour F, Sirvent N, et al. Deregulation of the platelet-derived growth factor B-chain gene via fusion with collagen gene COL1A1 in dermatofibrosarcoma protuberans and giant-cell fibroblastoma. Nat Genet 1997;15:95–98.
- 69.↑
Rutkowski P, Van Glabbeke M, Rankin CJ, et al. Imatinib mesylate in advanced dermatofibrosarcoma protuberans: pooled analysis of two phase II clinical trials. J Clin Oncol 2010;28:1772–1779.
- 70.↑
Navarrete-Dechent C, Mori S, Barker CA, et al. Imatinib treatment for locally advanced or metastatic dermatofibrosarcoma protuberans: a systematic review. JAMA Dermatol 2019;155:361–369.
- 71.↑
Kerob D, Porcher R, Verola 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;16:3288–3295.
- 72.↑
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;23:866–873.
- 73.↑
Rutkowski P, Dębiec-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;25:264–270.
