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Original Investigation
December 2016

Prognostic Factors, Treatment, and Survival in Dermatofibrosarcoma Protuberans

Author Affiliations
  • 1The Ronald O. Perelman Department of Dermatology, New York University School of Medicine, New York
JAMA Dermatol. 2016;152(12):1365-1371. doi:10.1001/jamadermatol.2016.1886
Key Points

Question  What is the prognostic significance of patient demographics, tumor characteristics, and/or treatment modalities for patients with dermatofibrosarcoma protuberans (DFSP)?

Findings  In this population-based cohort study of 3686 DFSP cases, older age, male sex, and tumor size were significantly associated with worse overall survival in a controlled analysis. Treatment modality did not influence overall survival; however, differences in patient characteristics affected the treatment received.

Meaning  Age at diagnosis, male sex, and DFSP tumor size seem to be important prognostic factors.

Abstract

Importance  There is limited information regarding the influence of patient demographics, tumor characteristics, and treatment type on the survival of patients with dermatofibrosarcoma protuberans (DFSP).

Objective  To assess prognostic factors and to evaluate the influence of treatment modality on overall survival of patients with DFSP.

Design, Setting, and Participants  We examined DFSP using data for 3686 patients with histologically confirmed cases of DFSP diagnosed between 1972 and 2012 from the 18 US regional registries of the National Cancer Institute’s Surveillance, Epidemiology, and End Results (SEER) Program, with linkage to demographic data from the US Census Bureau for median household income (MHI). The analysis was performed in February 2016.

Main Outcomes and Measures  The primary outcome measures were tumor characteristics, prognostic factors, and overall survival in months.

Results  There were 3686 cases of DFSP examined. Older age (hazard ratio [HR], 1.08; 95% CI, 1.06-1.10; P < .001), male sex (HR, 1.97; 95% CI, 1.09-3.55; P = .03), and tumor size (HR, 1.09; 95% CI, 1.01-1.18; P = .04) were significantly associated with poorer overall survival in a controlled analysis. Older age (odds ratio [OR], 1.01; 95% CI, 1.00-1.02; P = .01), male sex (OR, 1.95; 95% CI, 1.57-2.42; P < .001), and black race (OR, 1.78; 95% CI, 1.37-2.32; P < .001) were associated with larger (≥3.0 cm) tumors at presentation. Treatment modality did not influence overall survival; however, differences in patient characteristics affected the treatment received. Older age at presentation (OR, 1.02; 95% CI, 1.01-1.03; P =.01), black race (OR, 1.82; 95% CI, 1.13-2.92; P = .01), large tumor size (OR, 1.15; 95% CI, 1.09-1.21; P < .001), and head or neck location (OR, 4.63; 95% CI, 2.66-8.07; P <.001) increased the likelihood of a patient receiving surgery and radiation over surgery alone. In addition, white patients (OR, 0.51; 95% CI, 0.30-0.87; P=.01), women (OR, 0.53; 95% CI, 0.36-0.78; P <.001), and patients with a higher MHI (OR, 1.27; 95% CI, 1.11-1.46; P <.001) were more likely to receive Mohs micrographic surgery (MMS) over excision.

Conclusions and Relevance  Age at diagnosis, male sex, and DFSP tumor size appear to be important prognostic factors. Treatment modality did not significantly influence survival; however, patient and tumor characteristics influence treatment modality.

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