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• W2022925864 abstract "Purpose/Objective(s)ATRT is a rare pediatric brain tumor that was not widely recognized until the late 1990s. There is very limited information on its epidemiology, natural history, and factors associated with outcome. We used the National Cancer Institute SEER data-base to explore these issues.Materials/MethodsWe identified 101 patients younger than age 21 with intracranial ATRT in the SEER 1973 - 2007 dataset using the International Classification of Diseases for Oncology (ICD-0 - 3) code 9508. Overall survival (OS) rates were calculated using the Kaplan-Meier method. We used univariate and multivariate Cox proportional hazards models to identify factors associated with survival.ResultsMedian follow-up was 30 months (range, 1 - 85 months). Seventy-six patients were younger than age 3 at diagnosis. Thirty-six children (36%) received radiation therapy (RT). Ten patients had no surgery, 55 had a subtotal resection, and 36 had a gross total resection. Twenty-five patients had infratentorial tumors, and 18 patients had metastases at diagnosis. One- and 3-year OS rates for all patients were 44% and 27%, respectively. The median OS of patients treated with RT was not yet achieved at 85 months, which was markedly superior to the median survival of 6 months in patients who did not receive RT (log-rank p<0.001). The 3-year OS in the 20 children under age 3 with localized disease who received RT was 67%, compared to 8% in the 42 such children who were not treated with RT (log-rank p<0.001). Significant predictors of overall mortality on multivariate analysis were treatment with RT (HR 0.25, 95% CI 0.124 - 0.520, p<0.001) and the presence of metastatic disease at diagnosis (HR 2.26, 95% CI 0.133 - 4.49, p = 0.021). There was also a nearly significant trend for poorer survival for patients of non-white race (HR 1.85, 95% CI 0.98 - 3.50, p = 0.057). Age, tumor location, gender, and the extent of resection (gross total resection versus subtotal resection) did not impact survival.ConclusionsThis is the largest series of patients with ATRT yet reported. Treatment with RT and the presence of metastatic disease, but not age, were significant predictors of survival. Survival was very poor in children who did not receive RT, although it is unknown why RT was not recommended. If clinically feasible, radiation therapy should be considered even in young children for the greatest chance of disease control. Since the SEER database lacks data on chemotherapy and RT details, a prospective national registry of patients with ATRT is needed to further define appropriate treatment. Purpose/Objective(s)ATRT is a rare pediatric brain tumor that was not widely recognized until the late 1990s. There is very limited information on its epidemiology, natural history, and factors associated with outcome. We used the National Cancer Institute SEER data-base to explore these issues. ATRT is a rare pediatric brain tumor that was not widely recognized until the late 1990s. There is very limited information on its epidemiology, natural history, and factors associated with outcome. We used the National Cancer Institute SEER data-base to explore these issues. Materials/MethodsWe identified 101 patients younger than age 21 with intracranial ATRT in the SEER 1973 - 2007 dataset using the International Classification of Diseases for Oncology (ICD-0 - 3) code 9508. Overall survival (OS) rates were calculated using the Kaplan-Meier method. We used univariate and multivariate Cox proportional hazards models to identify factors associated with survival. We identified 101 patients younger than age 21 with intracranial ATRT in the SEER 1973 - 2007 dataset using the International Classification of Diseases for Oncology (ICD-0 - 3) code 9508. Overall survival (OS) rates were calculated using the Kaplan-Meier method. We used univariate and multivariate Cox proportional hazards models to identify factors associated with survival. ResultsMedian follow-up was 30 months (range, 1 - 85 months). Seventy-six patients were younger than age 3 at diagnosis. Thirty-six children (36%) received radiation therapy (RT). Ten patients had no surgery, 55 had a subtotal resection, and 36 had a gross total resection. Twenty-five patients had infratentorial tumors, and 18 patients had metastases at diagnosis. One- and 3-year OS rates for all patients were 44% and 27%, respectively. The median OS of patients treated with RT was not yet achieved at 85 months, which was markedly superior to the median survival of 6 months in patients who did not receive RT (log-rank p<0.001). The 3-year OS in the 20 children under age 3 with localized disease who received RT was 67%, compared to 8% in the 42 such children who were not treated with RT (log-rank p<0.001). Significant predictors of overall mortality on multivariate analysis were treatment with RT (HR 0.25, 95% CI 0.124 - 0.520, p<0.001) and the presence of metastatic disease at diagnosis (HR 2.26, 95% CI 0.133 - 4.49, p = 0.021). There was also a nearly significant trend for poorer survival for patients of non-white race (HR 1.85, 95% CI 0.98 - 3.50, p = 0.057). Age, tumor location, gender, and the extent of resection (gross total resection versus subtotal resection) did not impact survival. Median follow-up was 30 months (range, 1 - 85 months). Seventy-six patients were younger than age 3 at diagnosis. Thirty-six children (36%) received radiation therapy (RT). Ten patients had no surgery, 55 had a subtotal resection, and 36 had a gross total resection. Twenty-five patients had infratentorial tumors, and 18 patients had metastases at diagnosis. One- and 3-year OS rates for all patients were 44% and 27%, respectively. The median OS of patients treated with RT was not yet achieved at 85 months, which was markedly superior to the median survival of 6 months in patients who did not receive RT (log-rank p<0.001). The 3-year OS in the 20 children under age 3 with localized disease who received RT was 67%, compared to 8% in the 42 such children who were not treated with RT (log-rank p<0.001). Significant predictors of overall mortality on multivariate analysis were treatment with RT (HR 0.25, 95% CI 0.124 - 0.520, p<0.001) and the presence of metastatic disease at diagnosis (HR 2.26, 95% CI 0.133 - 4.49, p = 0.021). There was also a nearly significant trend for poorer survival for patients of non-white race (HR 1.85, 95% CI 0.98 - 3.50, p = 0.057). Age, tumor location, gender, and the extent of resection (gross total resection versus subtotal resection) did not impact survival. ConclusionsThis is the largest series of patients with ATRT yet reported. Treatment with RT and the presence of metastatic disease, but not age, were significant predictors of survival. Survival was very poor in children who did not receive RT, although it is unknown why RT was not recommended. If clinically feasible, radiation therapy should be considered even in young children for the greatest chance of disease control. Since the SEER database lacks data on chemotherapy and RT details, a prospective national registry of patients with ATRT is needed to further define appropriate treatment. This is the largest series of patients with ATRT yet reported. Treatment with RT and the presence of metastatic disease, but not age, were significant predictors of survival. Survival was very poor in children who did not receive RT, although it is unknown why RT was not recommended. If clinically feasible, radiation therapy should be considered even in young children for the greatest chance of disease control. Since the SEER database lacks data on chemotherapy and RT details, a prospective national registry of patients with ATRT is needed to further define appropriate treatment." @default.
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• W2022925864 title "Incidence, Outcomes, and Prognostic Factors in Children with Atypical Teratoid Rhabdoid Tumors (ATRT) Using the Surveillance Epidemiology and End Result (SEER) Data-Base" @default.
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