Dermatofibrosarcoma protuberans (DFSP) is a rare type of skin cancer. It begins in the connective tissue cells in the middle layer of the skin (dermis).
DFSP may look like a pimple at first or feel like a rough patch of skin on the skin. As it grows, lumps of tissue (protuberans) may form on the skin. It often occurs on the arms, legs and trunk. It grows slowly and rarely spreads beyond the skin.
For the diagnosis of dermatofibrosarcoma protuberans;
1. Skin Examination.
2. Skin biopsy.Tissue can be taken with punch, incisional or excisional biopsy. If DFS is clinically considered, excisional biopsy may not be preferred.
3.Display:Sometimes MRI or ultrasonography to determine the lesion margins can be done to see the size of the lesion and to assist in treatment planning.
Treatment
Treatment of dermatofibrosarcoma protuberans is surgical. In advanced cases, preoperative neoadjuvant and post-insufficient excision adjuvant treatments can be used.
Surgical treatment :It consists of WIDE LOCAL EXCISION and defect repair with a solid margin of 2-4 cm.
Surgery should be planned knowing that dermatofibrosarcoma protuberans tends to grow in an irregular manner which makes complete removal difficult. Mohs/or similar methods are recommended for surgical margin control. Since Mohs surgery takes a long time and requires an experienced center, Modified Mohs surgery (stepped surgery: make wide local excision, send it to pathology for tumor map, the result is sufficient surgical margin and the defect is closed preferably with skin graft. If the surgery is close, re-excision and repair of the defect) is preferred.
radiation therapy . It is not a primary treatment model. In cases where the surgical margin is insufficient, radiation therapy may be recommended.
Systemic drug therapy (Targeted therapy). Targeted drugs attack certain chemicals found in cancer cells. Targeted drug therapies block these chemicals, causing cancer cells to die. Some people with dermatofibrosarcoma protuberans have cancer cells that produce excess protein. A drug called imatinib (Gleevec) can target these cells and provide treatment benefits. Systemic treatment may be recommended in cases of insufficient surgical margin or recurrence.
