Outcomes of surgery for dermatofibrosarcoma protuberans

Eur J Surg Oncol. 2004 Apr;30(3):341-5. doi: 10.1016/j.ejso.2003.12.005.


Background: Dermatofibrosarcoma protuberans (DFSP) is a rare soft tissue sarcoma usually presenting as nodular cutaneous mass on the trunk and proximal extremity. The tumour grows slowly, typically over years. The standard treatment is wide local excision with at least a 3-cm margin. The local regional recurrence is up to 50%, emphasizing the need for wide margins for local control. A small fraction of DFSP may metastasize, but on histological examination such tumours have features of fibrosarcomas rather than DFSP.

Hypothesis: This study was done to review our experience of the time interval to recurrence of DFSP.

Design: A retrospective review was undertaken to identify patients with DFSP in our university teaching hospital.

Methods: All patients received their primary surgical treatment in our department between February 1968 and June 2001. Treatment consisted of wide local excision with margins of at least 3 cm. The chi-square test and Fisher's exact test were performed to determine the relationship between recurrence and clinicopathological variables. We evaluated the prognostic variables using the Kaplan-Meier method with log-rank comparison.

Results: The median follow-up period was 59 months. The 5 and 10-year disease-free survival (DFS) were 86 and 76%, respectively. The overall recurrence rate was 16.7%. The mean time to recurrence was 38+/-12 months (range 1-100 months). In 30% of those patients with recurrences, the local regional recurrence was after 5 years.

Conclusion: Wide local excision with good margins decreases local regional recurrences in patients with DFSP. Close surveillance is necessary even beyond 5 years because late recurrences occur.

MeSH terms

  • Adolescent
  • Adult
  • Aged
  • Child
  • Dermatofibrosarcoma / surgery*
  • Disease-Free Survival
  • Female
  • Humans
  • Male
  • Middle Aged
  • Neoplasm Recurrence, Local*
  • Retrospective Studies
  • Skin Neoplasms / surgery*
  • Surgical Procedures, Operative / methods*
  • Time Factors