Low-grade dermatofibrosarcoma protuberance - A rare case report

Dermatofibrosarcoma protuberans (DFSP) is a slow-growing, soft-tissue tumour of early or mid-adult life, affecting both the genders equally. The most common sites are soft tissue of the trunk (50 to 60%), followed by proximal extremities (20 to 30%) and the head and neck region (10 to 15%). Its metastatic potential is low though the local recurrence rate is high. Here, we report a case of a female patient with a large soft tissue growth located at the right cheek, chin and neck region. Local excision was done under the impression of a benign tumour such as lipoma or sebaceous cyst. Histological evaluation showed bland spindle cells arranged in a storiform pattern questioning the provisional diagnosis of the lesion. Further evaluation with the immunohistochemistry (IHC) panel confirmed the diagnosis of DFSP. Since it is a rare tumour of the head and neck region with non-alarming initial presentation and the potential for erroneous diagnosis as another lesion, we present a case of DFSP.