Dermatofibrosarcoma protuberans of the head and neck. A report of 16 cases

Arch Otolaryngol Head Neck Surg. 1993 Aug;119(8):891-6. 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.

Publication types

  • Review

MeSH terms

  • Adolescent
  • Adult
  • Aged
  • Aged, 80 and over
  • Child
  • Female
  • Fibrosarcoma / epidemiology*
  • Fibrosarcoma / mortality
  • Fibrosarcoma / surgery
  • Head and Neck Neoplasms / epidemiology*
  • Head and Neck Neoplasms / mortality
  • Head and Neck Neoplasms / surgery
  • Humans
  • Los Angeles / epidemiology
  • Male
  • Middle Aged
  • Neoplasm Recurrence, Local / epidemiology
  • Neoplasm Recurrence, Local / mortality
  • Neoplasm Recurrence, Local / surgery
  • Retrospective Studies
  • Salvage Therapy / statistics & numerical data
  • Skin Neoplasms / epidemiology*
  • Skin Neoplasms / mortality
  • Skin Neoplasms / surgery