Dermatofibrosarcoma protuberans coexisting in a patient with a vascular malformation-a rare coincidence

Dermatofibrosarcoma protuberans with fibrosarcomatous differentiation (DFSP-FS) is a rare soft tissue tumor with more aggressive behavior and it is not clear what causes this type of skin cancer. We describe the case of a 48-year-old woman who was born with a vascular malformation in the sternal region and presented suddenly with a soft tissue sarcoma (DFSP-FS) in the same territory. She was initially treated by embolization as the sarcoma was misdiagnosed but the tumor within 6 months seemed to be growing rapidly and reached a giant dimension with ulceration and required surgical intervention. The patient underwent a surgical removal of the mass but as the pathology report included a DFSP-FS with close margins,a second operation was required. A wide local excision was performed and reconstruction of defect by using bilateral pectoralis major muscle flaps and a full thickness skin graft from the abdominal wall. Post operatively the patient was treated with radiotherapy.