Recurrent dermatofibrosarcoma protuberans of lower anterior chest wall in a young male with early fibrosarcomatous change

Dermatofibrosarcoma protuberans

Abstract

Dermatofibrosarcoma protuberans (DFSP) is a rare, slow-growing tumor of fibro-histiocytic nature considered of low- to intermediate-grade malignant mesenchymal neoplasm. It mainly affects young and middle-aged male adults with an indolent, very slow but infiltrative growth pattern. It originates from the dermis, from which it often invades the subcutaneous tissue and accounting for 6% of all soft tissue sarcomas and less than 0.1% of all cutaneous malignancies. We report a case of 7 year old male child presented with recurrent DFSP which exhibited early fibrosarcomatous changes. It is a rare finding and challenging entity in histopathological diagnosis. This subset of patients requires wide excision with a 3 cm safety margin and may require adjunct radiotherapy.