From subtle to striking: A rare case of dermatofibroma protuberans and its clinical journey

Dermatofibrosarcoma protuberans (DFSP) is a rare type of soft tissue sarcoma, which is slow-growing. It arises from the dermal, subcutaneous layer and is locally aggressive. It commonly affects adults between 20 and 50 years old, with a slight male predominance. This case report emphasizes the case of a 35-year-old male patient who presented with a large, firm, nodular mass on his left upper arm that had been progressively increasing in size over 3 years. On inspection, the lesion measured approximately 13 cm in diameter, was large and lobulated with overlying red-brown discolouration. The patient reported movement discomfort, and mild itching was noted. Diagnostic evaluation was advised, including X-ray, Ultrasound, Computed Tomography and Magnetic resonance imaging. A provisional diagnosis of soft tissue sarcoma was made. An excisional biopsy was performed, and histopathological analysis demonstrated a storiform pattern of spindle-shaped cells infiltrating the dermis and subcutis, consistent with dermatofibrosarcoma protuberans. The patient underwent wide local excision with clear margins to prevent recurrence, and no metastasis was detected. Dermatofibrosarcoma protuberans is characterized by a high rate of local recurrence but a low risk of metastasis. Early diagnosis and complete surgical excision are treatment options for good prognosis. This case underscores the importance of diagnosing and treating Dermatofibrosarcoma protuberans without delays.