Dermatofibrosarcoma Protuberans of the Chest Wall: A Rare Case

Dermatofibrosarcoma protuberans (DFSP) is a rare, slow-growing, low-to-intermediate-grade soft tissue sarcoma, most commonly occurring as a plaque or nodule on the trunk. It can be locally recurrent but very rarely metastasizes. The treatment of choice is wide local excision (WLE). Molecular therapy is useful in those with a pathogenic COL1A1-PDGFB gene fusion. We present the case of a 38-year-old male with a slow-growing, mildly pruritic, 10 × 8 cm, skin-colored plaque with a nodular surface on the left side of the anterior chest wall. A skin biopsy showed spindle cells arranged in fascicles and foci with a storiform pattern in the dermis, extending into the subcutis, staining positive for CD34 and negative for S100. Based on these findings, a diagnosis of DFSP was made, and WLE was performed. This case highlights the diagnostic challenges posed by the indolent presentation of DFSP and the importance of early treatment with WLE. Awareness about this rare entity is essential to prevent misdiagnosis and delayed treatment.