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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 &gt;70 years had a poorer survival than those &lt;60 years ( P  &lt; 0.001). Compared to surgery with chemotherapy, surgery with chemoradiotherapy was linked to a 53% lower risk of mortality ( P  &lt; 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.

Changing incidence and survival of desmoplastic small round cell tumor in the USA