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August 1993

Dermatofibrosarcoma Protuberans of the Head and Neck: A Report of 16 Cases

Author Affiliations

From the Radiation Medical Group, San Diego, Calif (Dr Mark), and the Departments of Surgery (Division of Head and Neck Surgery) (Drs Bailet and Calcaterra), Radiation Oncology (Drs Tran and Poen), and Pathology (Dr Fu), UCLA Medical Center.

Arch Otolaryngol Head Neck Surg. 1993;119(8):891-896. doi:10.1001/archotol.1993.01880200097014

• 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.

(Arch Otolaryngol Head Neck Surg. 1993;119:891-896)

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