Background: Dermatofibrosarcoma protuberans (DFSP), an uncommon sarcoma of the dermis, is a low-grade, spindle-cell tumor that usually grows slowly and has a high local recurrence rate, but rarely metastasizes to distant sites. The diagnosis is based on histopathology and the standard treatment is wide surgical excision with tumor-free margins.
Case Report: Described is the case of a 75-year-old female who presented with a giant tumor on her upper right arm that had been increasing in size for a year. A biopsy provided the diagnosis of DFSP. Radical surgery was performed and the arm was disarticulated from the scapula.
Conclusion: Treatment for DFSP typically involves surgical excision; however, the size of the tumor in the present case was exceptionally large. Disarticulation offered the best prognosis.