Dermatofibrosarcoma protuberans: unmasking a rare clinical image

Abstract

Dermatofibrosarcoma protuberans (DFSP) is a rare, slow-growing, soft tissue tumor, with a prevalence of 0.8-4.5 cases per 1 million per year. As the name suggests, the tumor often involves the dermis and soft tissues, with pedunculation and spread in advanced stages. It accounts for between 1% and 6% of all soft tissue sarcomas and 18% of all cutaneous soft tissue sarcomas. Here, we report a case of a 72-year-old male, who presented in the dermatology outpatient department with an exophytic overgrowth over his right elbow in the last 15 years which has progressively increased in size with a noncontributory family history. Physical examination revealed a lobulated, soft, non-tender, and mobile mass over the right elbow. Surgical excision was performed under local anesthesia. The excised specimen measured 6 x 5.5 x 1.5 cm. The specimen was sent to the histopathology section of the Department of Pathology. Microscopically, H&E staining revealed predominantly spindle cells arranged in storiform pattern, cytoplasm is moderate and eosinophilic with oval to elongated, hyperchromatic nuclei. Intervening fibrous septa and inflammatory infiltrate are also seen. Since metastatic potential is common, follow-up every 6 months was advised.