Unusual presentation of dermatofibrosarcoma protuberans in a young female: a rare case report and literature review

Abstract

Introduction: Dermatofibrosarcoma protuberans (DFSP) is a rare, low-grade malignant cutaneous soft tissue sarcoma of fibroblastic origin, characterized by slow but infiltrative growth and a high propensity for local recurrence, while distant metastasis remains uncommon. The reported incidence ranges from 0.8 to 5 cases per million population per year, accounting for less than 0.1% of all malignant tumors and approximately 1% of soft tissue sarcomas. Case presentation: The author reports a case of 33-year-old woman with no known comorbidities who presented to neuro OPD with complaint of solitary, dark-brown lump, associated with occasional pain over the lateral aspect of the elbow, for 7–8 months which was expanding gradually. Further on, the histopathological report revealed DFSP; wide local excision with vascular flap with split thickness skin graft of left elbow was therefore performed. Conclusion: DFSP is a rare cutaneous sarcoma with a high risk of local recurrence but excellent overall prognosis. Presentation at the elbow is uncommon and may delay diagnosis. This case emphasizes the importance of histopathological confirmation and adequate surgical excision with appropriate reconstruction. Long-term surveillance remains essential to detect recurrence early.