Dermatofibrosarcoma protuberans of the head and neck. A report of 16 cases
R. J. Mark, J. W. Bailet, L. M. Tran, J. Poen, Y. S. Fu and T. C. Calcaterra
Radiation Medical Group, San Diego, Calif.
Dermatofibrosarcoma protuberans of the head and neck is a rare neoplasm.
Overall, sarcomas account for less than 1% of all head and neck
malignancies. Of 229 patients with sarcomas of the head and neck seen at
our institution between 1955 and 1988, 14 (7%) were assigned the diagnosis
of dermatofibrosarcoma protuberans after pathologic review. Two additional
cases have been seen since 1988, bringing the total to 16. All cases were
low-grade lesions. Follow-up ranged from 36 to 198 months, with a median of
114 months. Fifteen patients were managed with surgery alone, and nine
(60%) developed local recurrence. Eight were salvaged with further surgery.
There were no regional lymph node recurrences 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 were ultimately disease free after
salvage treatment. The overall 5-year survival rate was 93% (13/14). We
conclude from this series, and from a review of the literature, that wide
surgical resection achieving good margins offers excellent probability of
cure, and that radiation therapy is a reasonable alternative in patients
who have unresectable lesions or who are medically inoperable.
