title: Dermatofibrosarcoma protuberans of the head and neck. A report of 16 cases.
abstract: 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.
authors.affiliation: Radiation Medical Group, San Diego, Calif.
authors.forename: R J
authors.initials: RJ
authors.lastname: Mark
authors.forename: J W
authors.initials: JW
authors.lastname: Bailet
authors.forename: L M
authors.initials: LM
authors.lastname: Tran
authors.forename: J
authors.initials: J
authors.lastname: Poen
authors.forename: Y S
authors.initials: YS
authors.lastname: Fu
authors.forename: T C
authors.initials: TC
authors.lastname: Calcaterra
mesh_terms: D000293:Adolescent; D000328:Adult; D000368:Aged; D000369:Aged, 80 and over; D002648:Child; D005260:Female; D005354:Fibrosarcoma / Q000453:epidemiology* / Q000401:mortality / Q000601:surgery; D006258:Head and Neck Neoplasms / Q000453:epidemiology* / Q000401:mortality / Q000601:surgery; D006801:Humans; D015141:Los Angeles / Q000453:epidemiology; D008297:Male; D008875:Middle Aged; D009364:Neoplasm Recurrence, Local / Q000453:epidemiology / Q000401:mortality / Q000601:surgery; D012189:Retrospective Studies; D016879:Salvage Therapy / Q000706:statistics & numerical data; D012878:Skin Neoplasms / Q000453:epidemiology* / Q000401:mortality / Q000601:surgery