Transformed dermatofibrosarcoma protuberans: A series of nine cases and literature review

Abstract

The fibrosarcomatous variant of dermatofibrosarcoma protuberans (DFSP) represents an uncommon form of DFSP which has a higher risk of local recurrence and distant metastases than ordinary DFSP. The aim of our study is to investigate clinicopathologic characteristics, treatment modalities and prognostic factors of nine cases of transformed DFSP admitted in Salah Azaiez Institute between 2002 and 2009. They were five men and four women. Median age at diagnosis was 52 years (35–87 years). The lesions were located on the abdominal wall (three cases), the upper limb (two cases), the back (two cases), the lower limb (one case) and the chest wall (one case). Tumor size ranged from 25 mm to 150 mm. After diagnosis, six patients were treated by wide local excision with margins ⩾2 cm, two patients had local excision without defined margins and one patient had incomplete local excision. Three patients underwent radiotherapy because of either cramped or unknown limits. Local recurrence was diagnosed in 5 cases and distant metastasis occurred in one patient. Fibrosarcomatous DFSP exhibits more aggressive behavior than DFSP. Their similar clinical presentation requires histopathological differentiation for prognosis. Treatment is based on wide local excision, radiation and targeted therapy.

Keywords

• Dermatofibrosarcoma protuberans
• Fibrosarcoma
• Surgery