Dermatofibrosarcoma protuberans, lymphedema, and breast cancer: A rare association

Abstract

A woman with postmastectomy lymphedema presented with a progressive nodular swelling on the affected limb. The biopsy was reported as dermatofibrosarcoma protuberans (DFSP). There is only one other case in literature, where a truncal dermatofibrosarcoma was reported in association with breast cancer. Both tumors had the P53 mutation. In our patient, the breast cancer and the DFSP were both negative for the P53 mutation and it is possible that chronic lymphedema was causative in the formation of the DFSP. More evidence is needed to understand the etiopathogenesis of DFSP occurring in breast cancer patients.