DERMATOFIBROSARCOMA PROTUBERANS OF THE HEAD AND NECK - A REPORT OF 16CASES

Dermatofibrosarcoma protuberans of the head and neck is a rare neoplas m. Overall, sarcomas account for less than 1% of all head and neck mal ignancies. Of 229 patients with sarcomas of the head and neck seen at our institution between 1955 and 1988,14 (7%) were assigned the diagno sis of dermatofibrosarcoma protuberans after pathologic review. Two ad ditional cases have been seen since 1988, bringing the total to 16. Al l cases were low-grade lesions. Follow-up ranged from 36 to 198 months , with a median of 114 months. Fifteen patients were managed with surg ery alone, and nine (60%) developed local recurrence. Eight were salva ged with further surgery. There were no regional lymph node recurrence s or distant metastases. One patient was judged to be a poor surgical candidate and received primary radiation therapy. He died disease free 3 years after treatment. At last follow-up, 15 (94%) of 16 patients w ere ultimately disease free after salvage treatment. The overall 5-yea r survival rate was 93% (13/14). We conclude from this series, and fro m a review of the literature, that wide surgical resection achieving g ood margins offers excellent probability of cure, and that radiation t herapy is a reasonable alternative in patients who have unresectable l esions or who are medically inoperable.