Dermatofibrosarcoma protuberans: A rare presentation with lung and abdominal metastasis

Abstract

Dermatofibrosarcoma protuberans (DFSP) is an uncommon locally invasive, slow-growing tumor of the skin and the subcutaneous tissue. The skin lesion appears initially as a single, raised, red to bluish, firm cutaneous nodule or plaque with surrounding discoloration. It occurs usually at middle age with a male preponderance with an annual incidence of nearly 0.8 cases/million. The tumor occurs predominantly on the trunk followed by the proximal extremities and in the head and neck area. Even after wide surgical excision which is the standard treatment, it often recurs and becomes progressive with reported recurrence rate in the literature between 10% and 80% of cases. Distant metastasis is rarely observed with a rate of 4-6% and occurs mostly after repeated local recurrence. The lung is the most common site of distal metastasis. Metastasis to abdominal as well peri-intestinal site with serosal implant was rarely reported. Metastasis was histologically confirmed. We are reporting this case as a rare presentation of DFSP with distal multiple metastases to the lung and abdomen occurring 8 years after initial diagnosis and so emphasize on long-term regular follow-up.

Keywords

• Dermatofibrosarcoma protuberans
• metastasis
• recurrence
• swelling