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Objective. Clear cell sarcoma of the kidney (CCSK) is a lethal pediatric renal malignancy with poor prognosis. A prognostic nomogram needs to be established for overall survival (OS) prediction of patients with CCSK. Methods. Eligible 2588 CCSK patients (age 0–19) diagnosed between 2000 and 2017 were extracted from the Surveillance, Epidemiology, and End Results (SEER) database. Patients were randomized into training and validation cohorts (7 : 3). Independent prognostic factors were identified by univariate and multifactorial Cox regression analyses and used to construct a nomogram. Receiver operating characteristics (ROC) analysis, calibration curves, and decision curve analysis (DCA) were used to validate the nomogram. Moreover, a risk classification system was established based on the risk scores of the nomogram. Results. Cox analyses revealed that age, combined stage, and origin were most significant prognostic factors. Based on these prognostic factors, a nomogram was established for predicting 3- and 5-year OS of patients with CCSK. The area under the ROC curve (AUC) of 3- and 5-year OS was 0.733 and 0.728 in the training cohort, corresponding to 0.69 and 0.674 in the validation cohort. The C-index of calibration curves in the training and validation cohorts was 0.724 and 0.686. DCAs indicated the clinical utility of this nomogram. A risk classification system stratified CCSK patients into three different risk cohorts. The OS time of low-, intermediate-, and high-risk patients was 76, 68, and 65 months in the training cohort, corresponding to 69.5, 66, and 72 months in the validation cohort. Conclusion. A nomogram-based risk classification system has high accuracy for the prognostic prediction of CCSK.
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