The Epidemiology of Dermatofibrosarcoma Protuberans Incidence, Metastasis, and Death Among Various Population Groups: A SEER Database Analysis

J Am Acad Dermatol. 2024 Jun 20:S0190-9622(24)00956-3. doi: 10.1016/j.jaad.2024.05.088. Online ahead of print.


Background: Limited information exists regarding the epidemiology, metastasis, and survival of dermatofibrosarcoma protuberans (DFSP).

Objective: To measure DFSP incidence and assess metastasis and survival outcomes.

Methods: Incidence rate, overall and DFSP-specific survival outcomes for primary DFSP tumors contained in the Surveillance, Epidemiology, and End Results registry (SEER) were analyzed via quasi-poisson regression, Cox and competing risk analyses.

Results: DFSP incidence rate was 6.25 (95%CI, 5.93-6.57) cases per million person-years with significantly higher incidence observed among Black individuals than White individuals (8.74 vs 4.53). DSFP with larger tumor size (≥3 cm, Odds ratio,OR,2.24,95%CI,1.62-3.12,p <0.001) and tumors located on the head and neck (OR: 4.88, 95%CI, 3.31-7.18, p<0.001), and genitalia OR:3.16,95%CI:1.17-8.52, p value=0.023) were associated with significantly increased risk of metastasis whereas higher socioeconomic status (SES) was associated with significantly decreased risk of metastasis. Larger tumor size (≥3 cm), regardless of location, and age (≥60) were associated with significantly worse overall and cancer-specific survival.

Limitations: Retrospective design of SEER CONCLUSION: DFSP incidence is 2-fold higher among Black than White individuals. The risk of DFSP metastasis is significantly increased with tumor size ≥3 cm and tumors located on head and neck, and genitalia. Larger tumor size (≥ 3cm), regardless of location, and age (≥60) are the most important prognostic indicators for survival.

Keywords: DFSP; Dermatofibrosarcoma protuberans; cancer-specific survival; incidence; survival analysis.