Dermatofibrosarcoma protuberans: therapeutic challenges

M. E. Asuquo1, J. Omotoso2, G. Ebughe2
1Departments of Surgery, University of Calabar Teaching Hospital, Calabar, Nigeria
2Departments of Pathology, University of Calabar Teaching Hospital, Calabar, Nigeria

Tóm tắt

Dermatofibrosarcoma protuberans (DFSP) is an uncommon dermal neoplasm with proclivity for recurrence with little metastatic potential. Patients with histologic diagnosis of DFSP seen during a ten year period (January 2000 to December 2009) at the University of Calabar Teaching Hospital Calabar, Nigeria were studied. In total, 11 patients (5 males and 6 females) whose ages ranged from 17–52 years (mean 31.7 years) accounted for 6.8% of skin malignancies. The commonest site was the trunk 6 (54.5%), the head and neck ranked second, 3 (27.3%) followed by the lower limb 2 (18.2%). Eight (73%) patients presented with recurrent lesions. Follow up was poor (mean 6.8 months). DFSP is an uncommon tumour with most patients presenting with recurrent lesions. Follow up was poor; consequently, we advocate adjuvant radiotherapy and treatment with imatinib be included in patients treatment plan from the onset.

Tài liệu tham khảo

Lemm D, Mugge LO, Mentzel T, Hoffken K (2009) Current treatment options in dermatofibrosarcoma protuberans. J Cancer Res Clin Oncol 135(5):653–655 Asuquo ME, Umoh MS, Ebughe G (2007) Dermatofibrosarcoma protuberans: case reports. Ann Afr Med 6(2):80–83 Chen CJ, Siegel DM (2009) Dermatofibrosarcoma protuberans. eMedicine Dermatology updated Aug 17. Available at http://emedicine.medscape.com/article/110023 Chang CK, Jacobs IA, Salti GI (2004) Outcomes of surgery for dermatofibrosarcoma protuberans. Eur J Surg Oncol 30(3):341–345 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 Yu W, Tsoukas MM, Chapmann SM, Rosen JM (2008) Surgical treatment for dermatofibrosarcoma protuberans: the Dartmouth experience and literature review. Ann Plast Surg 60(3):288–293 Kaya G (2009) New molecular approaches in dermatofibrosarcoma protuberans. Rev Med Suisse 5(211):1519–1524 McArthur G (2007) Dermatofibrosarcoma protuberans: recent clinical progress. Ann Surg Oncol 14(10):2876–2886 Monnier D, Vidal C, Martin L, 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(10):1237–1242 Abbott JJ, Oliveira AM, Nascimento AG (2006) The prognostic significance of fibrosarcomatous transformation in dermatofibrosarcoma protuberans. Am J Surg Pathol 30(4):436–443 Ugurel S, Kortmann RD, Mohr P, et al (2005) Short German guidelines: dermatofibrosarcoma protuberans. J Dtsch Dermatol 53(1):76–83 Prieto VG, Reed JA, Shea CR (1994) CD34 immunoreactivity distinguishes between scar tissue and residual tumour in re-excisional specimens of dermatofibrosarcoma protuberans. J Cutan Pathol 21(4):324–329 DuBay D, Cimmino V, Lowe L, et al (2004) Low recurrence rate after surgery for dermatofibrosarcoma protuberans: a multidisciplinary approach from a single institution. Cancer 100(5):1008–1016 Lobropoulous SV, Razis ED (2007) Imatinib in the treatment of dermatofibrosarcoma protuberans. Biologics 1(4):347–353