Abstract
Ewing's sarcoma is a high-grade malignancy bone and soft tissue tumor that most commonly occurs in children and adolescents. Although the overall prognosis of Ewing's sarcoma has improved, the 5-year survival rate has not improved significantly. The study aimed to determine the risk factors independently associated with the prognosis of Ewing's sarcoma and to construct a nomogram to predict patient survival. Patients diagnosed with Ewing's sarcoma were collected from the Surveillance, Epidemiology, and End Results program database between 2004 and 2015 and further divided into training and validation cohort. Univariate and multivariate Cox regression analyses were used to identify meaningful independent prognostic factors. The nomogram was used to predict 3- and 5-year overall survival (OS) and cancer-specific survival (CSS). Finally, the nomogram was verified internally and externally through the training and validation cohorts, and the predictive capability was evaluated using the receiver operating characteristic (ROC) curve, C-index, and calibration curve and compared with that of the 7th TNM stage. A total of 1120 patients were divided into training (n = 713) and validation (n = 407) cohorts. Based on the multivariate analysis of the training cohort, a nomogram that integrated age, tumor size, primary site, N stage, and M stage was constructed (P < 0.05). The predicted C-indexes of OS and CSS of the training cohort were 0.744 (95% CI 0.717–0.771) and 0.743 (95% CI 0.715–0.770), respectively. However, the TNM stage had a C-index of 0.695 (95% CI 0.666–0.724) and 0.698 (95% CI 0.669–0.727) for predicting OS and CSS, respectively. The nomogram showed higher C-indexes than those in the TNM stage. Furthermore, the internal and external calibration curves showed good consistency between the predicted and observed values. Age, tumor size, primary site, N stage, and M stage are independent risk factors affecting the OS and CSS in Ewing’s sarcoma patients. Compared with the 7th TNM staging, the nomogram consisting of these factors was more accurate for risk assessment and survival prediction in patients with Ewing’s sarcoma, thus providing a novel reliable tool for risk assessment and survival prediction in Ewing’s sarcoma patients.
Highlights
Ewing’s sarcoma is a high-grade malignancy bone and soft tissue tumor that most commonly occurs in children and adolescents
We evaluated Ewing’s sarcoma (ES) patients registered in the SEER database from 2004 to 2015 and constructed a nomogram based on the clinicopathological data of these patients
The Kaplan–Meier survival curve showed that patients aged 0–17 years have a better prognosis than that in patients aged 18–59 years and ≥ 60 years (P < 0.001) (Fig. 1)
Summary
Ewing’s sarcoma is a high-grade malignancy bone and soft tissue tumor that most commonly occurs in children and adolescents. Tumor size, primary site, N stage, and M stage are independent risk factors affecting the OS and CSS in Ewing’s sarcoma patients. First proposed by James Ewing in 1921, it is the second most common primary bone and soft tissue malignancy in children and adolescents[2] It commonly occurs in the backbone of the long bones of the limbs and has a predilection for the femur, tibia, and humerus. Variables N Age 0–17 18–59 ≥ 60 Race Black White Other Gender Female Male Primary site Axial Extremity Other Tumor size ≤ 5 cm 6–10 cm > 10 cm Metastasis No Yes T stage T0 T1 T2 T3 Tx N stage N0 N1 Nx. and multi-drug chemotherapy have improved the 5-year overall survival (OS) rate of patients with localized ES from approximately 10% to nearly 75%5. The 5-year OS of metastasis patients is only 20–45%, depending on the location of m etastasis[4,7]
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