Dermatofibrosarcoma Protuberans: A Diagnostic and Management Conundrum—A Retrospective Study from a Tertiary Care Center in South India

DuBay D, Cimmino V, Lowe L, Johnson TM, Sondak VK (2004) Low recurrence rate after surgery for dermatofibrosarcoma protuberans: a multidisciplinary approach from a single institution. Cancer 100:1008–1016. https://doi.org/10.1002/cncr.20051

Article  PubMed  Google Scholar 

Menon G, Brooks J, Ramsey ML (2024) Dermatofibrosarcoma Protuberans. In: StatPearls [Internet]. Treasure Island (FL): StatPearls Publishing; 2025 Jan-. Available from: https://www.ncbi.nlm.nih.gov/books/NBK513305/

Wiesmueller F, Agaimy A, Perrakis A et al (2019) Dermatofibrosarcoma protuberans: surgical management of a challenging mesenchymal tumor. World J Surg Onc 17:90. https://doi.org/10.1186/s12957-019-1627-3

Article  Google Scholar 

Martin L, Piette F, Blanc P, Mortier L, Avril MF, Delaunay MM, Dréno B, Granel F, Mantoux F, Aubin F, Sassolas B, Adamski H, Dalac S, Pauwels C, Dompmartin A, Lok C, Estève E, Guillot B, French Group for Cutaneous Oncology (2005) Clinical variants of the preprotuberant stage of dermatofibrosarcoma protuberans. Br J Dermatol 153(5):932–6

Article  CAS  PubMed  Google Scholar 

Bogucki B, Neuhaus I, Hurst EA (2012) Dermatofibrosarcoma protuberans: a review of the literature. Dermatol Surg 38(4):537–551. https://doi.org/10.1111/j.1524-4725.2011.02292.x

Article  CAS  PubMed  Google Scholar 

Yu Q, Zhu Y, Huang R, Li Y, Song L, Zhang X et al (2022) Diagnosis and differential diagnosis of dermatofibrosarcoma protuberans: utility of high-resolution dynamic contrast-enhanced (DCE) MRI. Skin Res Technol 28:651–663. https://doi.org/10.1111/srt.13164

Article  PubMed  PubMed Central  Google Scholar 

Pretell-Mazzini J, Barton MD Jr, Conway SA, Temple HT (2015) Unplanned excision of soft-tissue sarcomas: current concepts for management and prognosis. J Bone Joint Surg Am 97(7):597–603. https://doi.org/10.2106/JBJS.N.00649

Article  PubMed  Google Scholar 

Khatri VP, Galante JM, Bold RJ, Schneider PD, Ramsamooj R, Goodnight JE Jr (2003) Dermatofibrosarcoma protuberans: reappraisal of wide local excision and impact of inadequate initial treatment. Ann Surg Oncol 10(9):1118–1122. https://doi.org/10.1245/aso.2003.03.581

Article  PubMed  Google Scholar 

Fletcher CD (2014) The evolving classification of soft tissue tumours-an update based on the new 2013 WHO classification. Histopathology 64:2–11. https://doi.org/10.1111/his.12267

Article  PubMed  Google Scholar 

Li Y, Wang C, Xiang B, Chen S, Li L, Ji Y. Clinical features, pathological findings and treatment of recurrent dermatofibrosarcoma protuberans. J Cancer 2017; 8(7):1319–1323. https://doi.org/10.7150/jca.17988. https://www.jcancer.org/v08p1319.htm

Rasheed AA, Barwad A, Dhamija E, Garg R, Pandey R, Shamim SA, Arun Raj ST, Rastogi S (2021) Advanced dermatofibrosarcoma protuberans: an updated analysis of cases from an Indian sarcoma clinic. Future Sci OA 30;7(9):FSO743. https://doi.org/10.2144/fsoa-2020-0198.

Ta RK, Banerjee SN (2015) Dermatofibrosarcoma protuberans: a rare presentation with lung and abdominal metastasis. Med J Dr. D.Y. Patil Univ 8(5):663–666. https://doi.org/10.4103/0975-2870.164949

Article  Google Scholar 

Fiore M, Miceli R, Mussi C, Lo Vullo S, Mariani L, Lozza L, Collini P, Olmi P, Casali PG, Gronchi A (2005) Dermatofibrosarcoma protuberans treated at a single institution: a surgical disease with a high cure rate. J Clin Oncol 23(30):7669–7675. https://doi.org/10.1200/JCO.2005.02.5122

Article  PubMed  Google Scholar 

Harati K, Lange K, Goertz O, Lahmer A, Kapalschinski N, Stricker I, Lehnhardt M, Daigeler A (2017) 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 15(1):5. https://doi.org/10.1186/s12957-016-1075-2

Article  PubMed  PubMed Central  Google Scholar 

National Comprehensive Cancer Network (2025) Dermatofibrosarcoma protruberans (Version 1.2025). https://www.nccn.org/professionals/physician_gls/pdf/dfsp.pdf

Lowe GC, Onajin O, Baum CL, Otley CC, Arpey CJ, Roenigk RK, Brewer JD (2017) 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 43(1):98–106. https://doi.org/10.1097/DSS.0000000000000910

Article  CAS  PubMed  Google Scholar 

Chen Y, Jiang G (2021) Association between surgical excision margins and outcomes in patients with dermatofibrosarcoma protuberans: a meta-analysis. Dermatol Ther 34(4):e14954. https://doi.org/10.1111/dth.14954

Article  PubMed  Google Scholar 

Hamid R, Hafeez A, Darzi AM, Zaroo I, Owais H, Akhter A (2013) Dermatofibrosarcoma protuberans: role of wide local excision. South Asian J Cancer 2:232–238

Article  PubMed  PubMed Central  Google Scholar 

Hamid R, Hafeez A, Darzi MA, Zaroo I, Rasool A, Rashid H (2013) Outcome of wide local excision in dermatofibrosarcoma protuberans and use of radiotherapy for marginpositive disease. Indian Dermatol Online J 4:936

Article  Google Scholar 

Parker TL, Zitelli JA (1995) Surgical margins for excision of dermatofibrosarcoma protuberans. J Am Acad Dermatol 32(2 Pt 1):233–236. https://doi.org/10.1016/0190-9622(95)90132-9

Article  CAS  PubMed  Google Scholar 

Rasheed AA, Barwad A, Dhamija E, Garg R, Pandey R, Shamim SA, Arun Raj ST, Rastogi S (2021) Advanced dermatofibrosarcoma protuberans: an updated analysis of cases from an Indian sarcoma clinic. Future Sci OA 7(9):FSO743. https://doi.org/10.2144/fsoa-2020-0198

Article  CAS  PubMed  PubMed Central  Google Scholar 

Rastogi S, Dhamija E, Barwad A, Aggarwal A, Sharma A, Panday R (2018) Advanced dermatofibrosarcoma protuberans treatment with imatinib: experience from a dedicated sarcoma medical oncology clinic in India. J Glob Oncol 4:1–7. https://doi.org/10.1200/JGO.18.00007.PMID:30085879;PMCID:PMC6223511

Article  PubMed  Google Scholar 

Bowne WB, Antonescu CR, Leung DH et al (2000) Dermatofibrosarcoma protuberans: a clinicopathologic analysis of patients treated and followed at a single institution. Cancer 88(12):2711–2720

Article  CAS  PubMed  Google Scholar 

Maghfour J, Genelin X, Olson J, Wang A, Schultz L, Blalock TW (2024) The epidemiology of dermatofibrosarcoma protuberans incidence, metastasis, and death among various population groups: a surveillance, epidemiology, and end results database analysis. J Am Acad Dermatol 91(5):826–833. https://doi.org/10.1016/j.jaad.2024.05.088

Article  PubMed  Google Scholar 

Marcoval J, Moreno-Vílchez C, Torrecilla-Vall-Llosera C, Muntaner-Virgili C, Sidelnikova DP, Sanjuán X, Penín RM (2024) Dermatofibrosarcoma protuberans: a study of 148 patients. Dermatology 240(3):487–493. https://doi.org/10.1159/000536172

Article  CAS  PubMed  Google Scholar 

Goldblum JR (1995) CD34 positivity in fibrosarcomas which arise in dermatofibrosarcoma protuberans. Arch Pathol Lab Med 119(3):238–241

CAS  PubMed  Google Scholar 

Palmerini E, Gambarotti M, Staals EL, Zanella L, Sieberova G, Longhi A, Cesari M, Bonarelli S, Picci P, Ruggieri P, Alberghini M, Ferrari S (2012) Fibrosarcomatous changes and expression of CD34+ and apolipoprotein-D in dermatofibrosarcoma protuberans. Clin Sarcoma Res 2(1):4. https://doi.org/10.1186/2045-3329-2-4.PMID:22587823;PMCID:PMC3351741

Article  CAS  PubMed  PubMed Central  Google Scholar 

Monnier D, Vidal C, Martin L, Danzon A, Pelletier F, Puzenat E, Algros MP, Blanc D, Laurent R, Humbert PH et al (2006) Dermatofibrosarcoma protuberans: a population-based cancer registry descriptive study of 66 consecutive cases diagnosed between 1982 and 2002. J Eur Acad Dermatol Venereol 20:1237–1242. https://doi.org/10.1111/j.1468-3083.2006.01780.x

Article  CAS  PubMed  Google Scholar 

Mentzel T, Beham A, Katenkamp D, Dei Tos AP, Fletcher CD (1998) Fibrosarcomatous (‘high-grade’) dermatofibrosarcoma protuberans: clinicopathologic and immunohistochemical study of a series of 41 cases with emphasis on prognostic significance. Am J Surg Pathol 22(5):576–587

Article  CAS  PubMed  Google Scholar 

Du K, Li J, Tang L et al (2019) Role of postoperative radiotherapy in dermatofibrosarcoma protuberans: a propensity score-matched analysis. Radiat Oncol 14:20. https://doi.org/10.1186/s13014-019-1226-z

Article  PubMed  PubMed Central  Google Scholar 

Fionda B, Loperfido A, Di Stefani A, Lancellotta V, Paradisi A, De Angeli M, Cappilli S, Rossi E, Caretto AA, Zinicola T, Schinzari G, Gentileschi S, Morganti AG, Rembielak A, Peris K, Tagliaferri L (2024) The role of postoperative radiotherapy in the management of dermatofibrosarcoma protuberans: a multidisciplinary systematic review. J Clin Med 13(6):1798. https://doi.org/10.3390/jcm13061798.PMID:38542022;PMCID:PMC10971559

Article  PubMed  PubMed Central  Google Scholar 

Han A, Chen EH, Niedt G, Sherman W, Ratner D (2009) Neoadjuvant imatinib therapy for dermatof

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