Desmoplastic small round cell tumor (DSRCT) is one of the rarest pediatric tumors and was only recently classified in 1989. Categorized under sarcomas, DSRCT under the microscopic is a fusion of a both a wilms and ewing sarcoma gene. Although known as a mostly surgical tumor, chemotherapy, radiation, and new treatments like immunotherapy are vital in the battle against this fatal disease. DSRCT is known mostly in the adolescent and young adult population, where the rate of occurrence is predominantly in males. In a retrospective cohort analysis published in 2014 from the Journal of Cancer Epidemiology, the 5-year survival rate was found to be just 33.3% (Lettieri, Garcia-Filion, Hingorani, 2014). Factors impacting survival rate are the overall oncology role to treatment and management.
Current studies show improved survival rates for DSRCT when multi-modality therapy over surgery alone. When high dose chemotherapy and radiation are added to the treatment plan, median survival rates more than doubled from 26.7 to 63.4 month (Hayes-Jordan et al., 2014). Current clinical trials are looking at the role of immunotherapy and antibody therapy in DSRCT. Some DSRCT tumors express a gene called GD2, a protein on the surface of its cell. The GD2 gene is also expressed on the surface of neuroblastoma cells where already proven antibody therapy has improved survival in those tumors. Current research looks into treating DSRCT with already produced antibodies for neuroblastoma (Dobrenkov, Ostrovnaya, Cheung, 2016). Some therapies include but are not limited to intraperitoneal 8H9, the inclusion of Avastin during cytoreduction, pazopanib, hyperthermic intraperitoneal chemotherapy, and more. The presentation will conclude with focus on future research and treatment options for this rare tumor.
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