To further improve survival in Ewing sarcoma development of risk- and response adaptive treatment strategies are necessary to allow decision making at different disease stages. Accurate survival estimations are challenging. We developed and validated an easy-to-use survival estimation tool for EwS, based on age, volume, primary tumor localization and disease extent. Furthermore, we show that during the course of treatment survival changes as more information becomes available.
The model presented is based on a cohort of 1314 EwS patients with uniformity in diagnostics and treatment and availability of all relevant prognostic factors. The provided flowcharts are easy-to-use and based on assessable variables. The 13 prognostic groups provide detailed insight in expected survival and could assist in fine-tuning individual treatment. The prognostic groups were narrowed down to 5 risk categories (A-E) based on clinical expertise. The risk categories defined on clinical criteria are consistent with cross-validated risk categories defined on predicted 5-year survival probability. The information gained after surgery offers a second time-point for multidisciplinary decision-making, at this point histological response is an strong additional prognostic factor for OS.
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