Background : Dermatofibrosarcoma protuberans (DFSP) is a rare, slow-growing cutaneous sarcoma with a high risk of local recurrence if incompletely excised. Although its metastatic potential is low, late local recurrences may occur, underscoring the need for long-term surveillance. Case presentation : We report the case of an 81-year-old woman who developed a late local recurrence of DFSP in the left scapular region, 18 years after initial excision. Clinical examination revealed a polylobulated cutaneous mass without lymphadenopathy. Histopathology demonstrated a storiform spindle-cell proliferation infiltrating the dermis and subcutaneous tissue, with diffuse CD34 positivity. Surgical excision revealed tumor persistence at the inferior lateral margin. The patient was referred for re-excision and reconstructive surgery. Discussion : This case illustrates the potential for very late recurrence of DFSP, even decades after primary treatment. Complete surgical excision with wide margins or Mohs micrographic surgery remains the cornerstone of management. Radiotherapy may be considered in unresectable cases or when re-excision is not feasible. Targeted therapy with imatinib represents an option for advanced or metastatic disease harboring the COL1A1–PDGFB fusion gene. Conclusion : DFSP requires lifelong follow-up due to its potential for late recurrence. Achieving negative surgical margins is critical to prevent relapse, and a multidisciplinary approach is essential in recurrent or complex cases.
Benoumrhar et al. (Fri,) studied this question.
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