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Comparison of clinical features and outcomes in patients with extraskeletal vs skeletal Ewing sarcoma: an SEER database analysis of 3,178 cases.

ABSTRACT: Background:The clinicopathological characteristics, outcomes and prognostic factors of primary extraskeletal Ewing sarcoma (EES) remained insufficiently explored. We aimed to examine these aspects and compared the same with skeletal Ewing sarcoma (SES). Patients and methods:We identified Ewing sarcoma, peripheral primitive neuroectodermal tumors or Askin tumor patients who were registered in the Surveillance, Epidemiology, and End Results database from 1973 to 2014. Clinicopathological features were assessed by using Fisher's exact tests. Cancer-specific survival (CSS) and overall survival (OS) were estimated by using the Kaplan-Meier method and the Cox proportional hazards model. Prognostic factors were identified by multivariate Cox regression analysis. Results:The age of patients with EES was diagnosed to be higher and they were more likely to be female (46.1% vs 36.2%; P<0.001), have tumor <10 cm (49.8% vs 35.4%; P<0.001), have regional node involvement (5.4% vs 1.0%; P<0.001) and receive surgery (69.1% vs 53.8%; P<0.001) compared to patients with skeletal tumors. Metastatic status did not differ by origin. Kaplan-Meier analysis showed that the origin had significant difference in CSS and OS among patients aged 0-19 years and with metastatic stage at presentation, but not in patients aged 20-39, ?40 years and with no-metastatic stage. A Cox multivariable model controlling for differences between groups confirmed inferior survival for patients with EES. Age, tumor size, tumor stage and surgery were the most important factors significantly influencing both CSS and OS in the EES and SES patients. Race, year of diagnosis and tumor site were associated with CSS and OS among patients with SES, but failed in EES. Conclusion:The clinicopathological characteristics, outcomes and prognostic factors differed among patients with EES compared to patients with SES. Extraskeletal origin was an unfavorable prognostic factor.

SUBMITTER: Jiang S

PROVIDER: S-EPMC6260126 | biostudies-other | 2018

REPOSITORIES: biostudies-other

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Comparison of clinical features and outcomes in patients with extraskeletal vs skeletal Ewing sarcoma: an SEER database analysis of 3,178 cases.

Jiang Sujing S Wang Guannan G Chen Jieyu J Dong Ying Y

Cancer management and research 20181123

<h4>Background</h4>The clinicopathological characteristics, outcomes and prognostic factors of primary extraskeletal Ewing sarcoma (EES) remained insufficiently explored. We aimed to examine these aspects and compared the same with skeletal Ewing sarcoma (SES).<h4>Patients and methods</h4>We identified Ewing sarcoma, peripheral primitive neuroectodermal tumors or Askin tumor patients who were registered in the Surveillance, Epidemiology, and End Results database from 1973 to 2014. Clinicopatholo ...[more]

PMID: 30538569

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Project description:Ewing sarcoma is the second most common osseous disease in children and adolescents. It presents with a poor prognosis due to the high degree of malignancy and distant metastasis. In order to predict the disease prognosis and investigate a suitable therapeutic strategy for Ewing sarcoma, the present study aimed to describe the clinical characteristics, and to construct and validate nomograms for patients with non-metastatic Ewing sarcoma. A total of 627 cases of non-metastatic Ewing sarcoma were retrospectively collected from the Surveillance, Epidemiology, and End Results database between 2005 and 2014. Survival analysis and a machine learning model were used to identify independent prognostic variables and establish nomograms to estimate overall survival (OS) and cause-specific survival (CSS). The nomograms were bootstrap internally validated and externally validated using non-metastatic Ewing sarcoma cases from the First Affiliated Hospital of Zhengzhou University. The accuracy was also assessed by comparing with current American Joint Committee on Cancer (AJCC) staging systems. The total series consisted of 627 patients with non-metastatic Ewing sarcoma with a mean age of 20.14 years. Age, tumor extension, sex, International Classification of Diseases for Oncology, 3rd Edition histology, surgery and chemotherapy were identified as independent risk factors for OS and CSS. The aforementioned outcomes were incorporated to construct the nomograms, and the concordance indices (C-indices) for internal validation of OS and CSS prediction were 0.791 and 0.813, which were higher than those for AJCC sixth edition (OS, 0.531; CSS, 0.534) and seventh edition (OS, 0.547; CSS, 0.561), while the C-indices for external validation of OS and CSS prediction were 0.834 and 0.825, respectively. In conclusion, age, sex, tumor extension and surgery were independent prognostic factors for both OS and CSS. In addition, with regard to OS, the Ewing sarcoma subtype was a poor factor and chemotherapy was a favorable one. Nomograms based on reduced Cox models attained a satisfactory accuracy in predicting the survival of patients with non-metastatic Ewing sarcoma and could assist clinicians in evaluating survival more accurately.

Project description:BackgroundHepatoblastoma (HB), hepatocellular carcinoma (HCC), and embryonal sarcoma (ES) are the three main types of liver tumors in children and adolescents. At present, epidemiological knowledge and predictors of these three liver tumor types in multi-ethnic populations are limited. This study aimed to outline the clinical features and construct a prognostic nomogram for these tumors, which can contribute to the prediction of dynamic overall survival probability during the follow-up period.MethodsA total of 1,122 patients liver tumor patients between 2000 to 2019 in Surveillance, Epidemiology, and End Results (SEER) database were enrolled for the current study, and separated into 824 HB, 219 HCC, and 79 ES according to the type of pathology. Independent prognostic factors were screened by univariate and multivariate Cox regression analysis, and a prognostic nomogram was constructed for overall survival. The accuracy and discriminative abilities of the nomogram were evaluated by concordance index as well as time-dependent receiver operating characteristic curves and calibration curves.ResultsRace (P=0.0016), surgery [hazard ratio (HR): 0.1021, P<0.001], and chemotherapy (HR: 0.27, P=0.00018) are independent prognostic factors for hepatoblastoma. Pathological tissue grading (P=0.00043), tumor node metastasis (TNM) staging (P=0.00061), and surgery are independent prognostic factors for hepatocellular carcinoma. Household income and surgery (HR: 0.1906, P<0.001) are independent prognostic factors for embryonal sarcoma. All of these prognostic factors are significantly associated with prognosis. A nomogram consisting of these variables was established, which showed a good concordance index (0.747, 0.775, and 0.828 in hepatoblastoma, hepatocellular carcinoma, and embryonal sarcoma, respectively). Also, the 5-year area under curve (AUC) of the nomogram were 0.738, 0.812, and 0.839 in hepatoblastoma, hepatocellular carcinoma, and embryonal sarcoma, respectively. In the calibration diagram, an optimal agreement between the nomogram-predicted and actual observed survival was evident.ConclusionsWe developed an effective prognostic nomogram for overall survival prediction in hepatoblastoma, hepatocellular carcinoma, and embryonal sarcoma in children and adolescent patients, which will further benefit the assessment of long-term outcomes.

Dataset Information

Comparison of clinical features and outcomes in patients with extraskeletal vs skeletal Ewing sarcoma: an SEER database analysis of 3,178 cases.

Publications

Comparison of clinical features and outcomes in patients with extraskeletal vs skeletal Ewing sarcoma: an SEER database analysis of 3,178 cases.

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