The incidence and prognosis of desmoplastic small round cell tumor (DSRCT) is inadequately understood. Survival analysis for DSRCT has not been investigated in a population-based study. We conducted a retrospective cohort study using the Surveillance, Epidemiology, and End Results (SEER) 9 Registry (1975-2018). Annual percent changes in incidence were estimated using SEER*Stat, and risk ratios were estimated using Poisson regression. Cox regression models were constructed to estimate the hazard ratio for survival at 5 years. The incidence rate of DSRCT has been rising in the last two decades. Men had a higher age-adjusted incidence rate, and nonmetropolitan counties had a higher incidence rate than metropolitan counties. Blacks had a higher risk of being diagnosed with DSRCT than whites. The observed survival at 12, 36, and 60 months was 81%, 39.9%, and 23.4%, respectively. Those >70 years had a poorer survival than those <60 years (P < 0.001). Compared to surgery with chemotherapy, surgery with chemoradiotherapy was linked to a 53% lower risk of mortality (P < 0.001). We conclude that the DSRCT incidence has been increasing since 2000 with a white male predominance. Gender doesn't affect survival in DSRCT, and surgery combined with chemoradiotherapy improves survival compared to surgical management with chemotherapy alone.
Keywords: DSRCT; Desmoplastic small round cell tumor; SEER; incidence; prognosis; survival.
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