Dermatofibrosarcoma protuberans of the head and neck

Abstract

To elucidate the natural history of dermatofibrosarcoma protuberans of the head and neck, 17 cases were reviewed and compared with 92 others contained within the English literature. The prototypic case is seen as a firm, solitary, slowly enlarging, asymptomatic, cutaneous nodule between 2 and 5 cm in size and of two years' duration. It is more common in men and in persons aged between 30 and 40 years. The scalp and neck-supraclavicular fossa are the most common sites, accounting for half of the cases. Approximately 50% to 75% of the patients experience local recurrence after initial surgery and, of these, half to three fourths manifest within three years of treatment. Metastases, seen in no more than 6% of all cases, are almost invariably preceded by two or more local recurrences and occur primarily through the bloodstream to the lungs and bones and less often to regional lymph nodes. Prognostic factors, differential diagnosis, and histogenesis are included.