Recurrent Dermatofibrosarcoma Protruberance over Shoulder: An Unresolved Problem
International Research Journal of Oncology,
Introduction: We are reporting a case of recurrent Dermatofibrosarcoma protuberans (DFSP) over right shoulder in a 36 year young lady who underwent re-excision with secondary healing. DFSP is common in both, men and women with a slight male predominance. They are characterized by a unique translocation of chromosome i.e. t(17;22) (q22;q13). Majority of DFSPs are low grade (85-90 %) while the rest contain a high-grade sarcomatous component (DFSP-FS) and are considered to be intermediate-grade sarcomas. Trunk and proximal extremities are the most common location, usually on the chest and shoulders. Persistently recurring tumors have an increased risk for transformation into a more malignant form (DFSP-FS). Metastases to regional lymph nodes are extremely rare and distant hematogenous metastases are even rarer. Adequate lateral and deeper margin resection is necessary for excision of resectable primary DFSP and team approach is must for tackling recurrent and metastatic DFSP.
- Recurrent DFSP
- secondary healing
- hematogenous metastases
- surgical excision
How to Cite
Llombart B, Serra-Guillén C, Monteagudo C, et al. Dermatofibrosarcoma protuberans: A comprehensive review and update on diagnosis and management. Semin Diagn Pathol. 2013;30:13.
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.
Yagi Y, Ueda K, Maruyama S, Noborio R. Bednar tumor: a report of two cases. JDermatol. 2004;31:484.
Dupree WB, Langloss JM, Weiss SW. Pigmented dermatofibrosarcoma protuberans (Bednar tumor). A pathologic, ultrastructural, and immunohistochemical study. Am JSurg Pathol. 1985;9:630.
Stacchiotti S, Pedeutour F, Negri T, et al. Dermatofibrosarcoma protuberans-derived fibrosarcoma : clinical history, biological profile and sensitivity to imatinib. Int J Cancer. 2011;129:1761.
Love WE, Keiler SA, Tamburro JE, et al. Surgical management of congenital dermatofibrosarcoma protuberans. J Am Acad Dermatol. 2009;61:1014.
Thornton SL, Reid J, Papay FA, Vidimos AT. Childhood dermatofibrosarcoma protuberans: role of preoperative imaging. J Am Acad Dermatol. 2005;53:76.
Weinstein JM, Drolet BA, Esterly NB, et al. Congenital dermatofibrosarcoma protuberans: Variability in presentation. Arch Dermatol. 2003;139:207.
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.
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.
National Comprehensive Cancer Network (NCCN). NCCN clinical practice guidelines in oncology.
(Accessed on October 14, 2020).
Saiag P, Grob JJ, Lebbe C, et al. Diagnosis and treatment of dermatofibrosarcoma protuberans. European consensus-based interdisci-plinary guideline. Eur J Cancer. 2015;51: 2604.
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.
Paradisi A, Abeni D, Rusciani A, et al. Dermatofibrosarcoma protuberans: Wide localexcision vs. Mohs micrographic surgery. Cancer Treat Rev. 2008;34: 728.
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 of74 primary cases. Br J Dermatol. 2015;172:1303.
Fiore M, Miceli R, Mussi C, et al. Dermatofibrosarcoma protuberans treated at a single institution: a surgical disease with a high cure rate. J Clin Oncol 2005; 23:7669.
Mavili ME, Gursu KG, Gokoz A. Dermatofibrosarcoma with lymph node involvement. Ann Plast Surg. 1994;32:438.
Lemm D, Mügge LO, Mentzel T, Höffken K. Current treatment options in dermatofibrosarcoma protuberans. J Cancer Res Clin Oncol. 2009;135:653.
Sandberg AA, Bridge JA. Updates on the cytogenetics and molecular genetics of bone and soft tissue tumors. Dermatofibrosarcoma protuberans and giant cell fibroblastoma. Cancer Genet Cytogenet. 2003;140:1.
McArthur GA. Dermatofibrosarcoma protuberans: A surgical disease with a molecular savior. Curr Opin Oncol. 2006;18:341.
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.
Domanski HA, Gustafson P. Cytologic features of primary, recurrent, and metastatic dermatofibrosarcoma protuberans. Cancer. 2002;96:351.
Klijanienko J, Caillaud JM, Lagacé R. Fine-needle aspiration of primary and recurrent dermatofibrosarcoma protuberans. Diagn Cytopathol. 2004;30:261.
Haycox CL, Odland PB, Olbricht SM, Piepkorn M. Immunohistochemical characterization of dermatofibrosarcoma protuberans with practical applications for diagnosis and treatment. J Am Acad Dermatol. 1997;37:438.
Abenoza P, Lillemoe T. CD34 and factor XIIIa in the differential diagnosis of dermatofibroma and dermatofibrosarcoma protuberans. Am J Dermatopathol. 1993;15:429.
Rutgers EJ, Kroon BB, Albus-Lutter CE, Gortzak E. Dermatofibrosarcoma protuberans: treatment and prognosis. Eur J Surg Oncol. 1992;18:241.
Terrier-Lacombe MJ, Guillou L, Maire G, et al. Dermatofibrosarcoma protuberans, giant cell fibroblastoma, and hybrid lesions in children: Clinicopathologic comparative analysis of 28 cases with molecular data--a study from the French Federation of Cancer Centers Sarcoma Group. Am J Surg Pathol. 2003; 27:27.
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: 1118.
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; 20:1237.
Yu W, Tsoukas MM, Chapman SM, Rosen JM. Surgical treatment for dermatofibrosarcoma protuberans: the Dartmouth experience and literature review.Ann Plast Surg. 2008;60:288.
Fields RC, Hameed M, Qin LX, et al. Dermatofibrosarcoma protuberans (DFSP): Predictors of recurrence and the use of systemic therapy. Ann Surg Oncol. 2011;18:328.
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;67:1315.
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.
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.
Williams N, Morris CG, Kirwan JM, et al. Radiotherapy for dermatofibrosarcoma protuberans. Am J Clin Oncol. 2014;37:430.
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.
Navarrete-Dechent C, Mori S, Barker CA, et al. Imatinib Treatment for Locally Advancedor Metastatic Dermatofibrosarcoma Protuberans: A Systematic Review. JAMA Dermatol. 2019;155:361.
Stacchiotti S, Pantaleo MA, Negri T, et al. Efficacy and Biological Activity of Imatinib in Metastatic Dermatofibrosarcoma Protuberans (DFSP). Clin Cancer Res. 2016;22:837.
Kérob D, Porcher R, Vérola 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.
Abstract View: 107 times
PDF Download: 63 times