Desmoplastic small round cell tumor: Analysis of surveillance, epidemiology, and end results (SEER) database 1975 to 2018.

Abstract

e23540 Background: Desmoplastic small round cell tumor (DSRCT) is a rare and aggressive soft tissue sarcoma with a poor prognosis. The incidence and prognosis of patients with DSRCT have been inadequately understood. Survival analysis for DSRCT has been sparsely investigated in a population-based study. Methods: We conducted a retrospective cohort study using the SEER 9 Registry (1975-2018). Site recode International Classification of Diseases for Oncology (ICD-O-3) 8806/3 was used via SEER*Stat software to select DSRCT cases. SEER*Stat software estimated annual percent changes, and Poisson regression estimated risk ratios. Cox regression models were constructed to estimate the hazard ratio for survival at five years. The period-specific rates were portrayed as rate per 1,000,000. Results: The male and female age adjusted DSRCT incidence rates were 0.22 and 0.05, respectively (p < 0.05). Incidence rate of DSRCT has been rising in the last two decades from 1975–2000 to 2001-2018 (0.013 vs. 0.31,p < 0.05). Males have a higher age adjusted DSRCT incidence rate (0.22 vs. 0.05,p < 0.05), and non-metropolitan counties had a higher incidence rate than metro counties (1.14 vs. 0.14,p < 0.05). The observed survival at 12, 36, and 60 months was 81%, 39.9%, and 23.4%, respectively. There was no significant difference in survival between females and males. Ages greater than 70 years had poor survival compared to ages less than 60 years (p < 0.001). Compared to surgery with chemotherapy, surgery combined with radiotherapy and chemotherapy was associated with a 62% lower risk of mortality (p< 0.001). Conclusions: This study reveals that DSRCT incidence has been increasing since 2000, is more common in white males. Gender may not affect survival in DSRCT, and surgery combined with chemoradiotherapy is associated with improved survival outcomes compared to surgical management with chemotherapy alone. Further comparative data would be beneficial to confirm reported findings.[Table: see text]