scholarly journals Clinical outcome of patients with recurrent or refractory localized Ewing's sarcoma family of tumors: A retrospective report from the Japan Ewing Sarcoma Study Group

2021 ◽  
Author(s):  
Katsutsugu Umeda ◽  
Takako Miyamura ◽  
Kenji Yamada ◽  
Hideki Sano ◽  
Ako Hosono ◽  
...  
1997 ◽  
Vol 2 (3) ◽  
pp. 180-184 ◽  
Author(s):  
Toshifumi Ozaki ◽  
Winfried Winkelmann ◽  
Normann Willich ◽  
Herbert Jürgens

2010 ◽  
Author(s):  
Christopher A. Scannell ◽  
Diana Abdueva ◽  
Lingyun Ji ◽  
Cornelia von Levetzow ◽  
Richard Sposto ◽  
...  

1998 ◽  
Vol 16 (11) ◽  
pp. 3628-3633 ◽  
Author(s):  
A Craft ◽  
S Cotterill ◽  
A Malcolm ◽  
D Spooner ◽  
R Grimer ◽  
...  

PURPOSE To investigate the possibility that the substitution of ifosfamide for cyclophosphamide therapy for Ewing's sarcoma will improve survival over that seen in the first United Kingdom Children's Cancer Study Group (UKCCSG) Ewing's tumor study (ET-1). PATIENTS AND METHODS Between 1987 and 1993,243 patients (138 men or boys) were entered onto the study. The median age was 13.5 years (range, 1.5 to 27 years). The median follow-up was 58 months. Chemotherapy included four courses of vincristine 2 mg/m2; ifosfamide 9 g/m2; and doxorubicin 60 mg/m2 administered every 3 weeks. Treatment of the primary tumor was with surgery and/or radiotherapy followed by ifosfamide 6 g/m2; doxorubicin 60 mg/m2; and vincristine 2 mg/m2; with actinomycin D 1.5 mg/m2 substituted for doxorubicin after a total dose of 420 mg/m2. RESULTS Two hundred one patients had no metastases. One hundred eighteen patients had tumors of the axial skeleton and 125 patients had limb primary tumors. The major toxicities were hematologic and infective, but there were no toxic deaths. The overall survival rate was 62% (95% confidence interval [CI], 56 to 69) and relapse-free survival (RFS) 56% (95% CI, 49 to 62). For those with no metastases at diagnosis, the RFS rate was 62% and for those with metastases, 23%. Multivariate analysis showed age and site to have a significant effect on RFS. Pelvic sites had the worst RFS rate of 41%; other axial sites, 55%; and extremity tumors, 73%. Age younger than 10 years had an RFS rate of 86% versus 55% for older patients. The local relapse rate for axial tumors was 20% and for limb primary tumors was 2.4%. CONCLUSION The 5-year survival rate of 62% is improved compared with the 44% survival rate achieved in ET-1. This is probably caused by the use of higher doses of ifosfamide compared with relatively low doses of cyclophosphamide in ET-1.


2020 ◽  
Author(s):  
Jie Jiang ◽  
Chong Liu ◽  
Guoyong Xu ◽  
Tuo Liang ◽  
Chaojie Yu ◽  
...  

Abstract Background: Ewing's sarcoma (ES) is the second most prevalent malignancy among bone tissue tumors, and there is no adequate prognosis biomarker. The protein encoded by CCT6A is a molecular chaperone. Early studies have suggested that CCT6A is involved in the development of many cancers, however, there is no clear evidence of a role for CCT6A in ES.Methods: In this study, we performed a bioinformatics analysis of 32 Ewing sarcoma specimens from the GSE17618 dataset for differences in gene expression and overall survival, event-free survival, and gene expression in different subgroups. Results: After three screenings, we identified CCT6A as highly correlated with Ewing's sarcoma prognosis. Survival analysis showed low overall survival (OS) for CCT6A high expression (P=0.024). On the other hand, Cox regression analysis showed that CCT6A expression, event-free survival (EFS), and age were strongly associated with the prognosis of Ewing sarcoma, identified as independent poor prognostic biomarkers. (CCT6A: P=0.015; Age: P-value=0.026; EFS: P-value=0.001). Conclusion: The expression level of CCT6A is strongly associated with the prognosis of Ewing's sarcoma. High expression of the CCT6A gene may serve as a biomarker for poor prognosis in patients with Ewing's sarcoma.


2021 ◽  
Author(s):  
wang chengwei

Abstract Background: Few models have been used to estimate the survival rate of patients metastatic Ewing sarcoma of bone are scarce. We aimed to develop nomograms for predicting 3-, 5-year survival for these patients.Methods: We extracted 686 cases of metastatic Ewing's sarcoma diagnosed between 1973 and 2016 from the Surveillance, Epidemiological and End Results (SEER) database. Univariate and multivariate Cox analysis were used to determine independent prognostic factors. The nomograms are based on the results of multivariate Cox analysis. We also evaluate the performance of these prediction models through the analysis of time-dependent receiver operating characteristic curve, concordance index, calibration curve and decision curve.Results: Age, surgery, tumor size, treatment method and chemotherapy were considered to be important predictors of overall survival of bone metastatic Ewing's sarcoma. Based on these factors, the nomogram models were established and verified internally. These models have good identification and calibration characteristics. A risk classification system based on nomogram has also been constructed to promote risk stratification of metastatic Ewing's sarcoma and to optimize clinical management.Conclusions: We developed the first nomograms and corresponding risk classification system to predict the survival of patients with bone metastatic Ewing's sarcoma. These easy-to-use tools can help oncologists and surgeons make accurate survival assessments.


2000 ◽  
Vol 18 (17) ◽  
pp. 3108-3114 ◽  
Author(s):  
S.J. Cotterill ◽  
S. Ahrens ◽  
M. Paulussen ◽  
H.F. Jürgens ◽  
P.A. Voûte ◽  
...  

PURPOSE: To further elaborate on prognostic factors for Ewing’s sarcoma of bone and to document improvements in relapse-free survival (RFS) and trends in local therapy over the study period (1977 to 1993). PATIENTS AND METHODS: A retrospective analysis was performed on a combined Gesellschaft Für Pädiatrische Onkologie und Hämatologie/Cooperative Ewing Sarcoma Study and United Kingdom Children’s Cancer Study Group/Medical Research Council data set of 975 patients registered with the respective trial offices before the current collaborative European Intergroup Cooperative Ewing’s Sarcoma Study trial. Both groups independently undertook studies with similar chemotherapy during the period. RESULTS: The key adverse prognostic factor is metastases at diagnosis (5-year RFS, 22% of patients with metastases at diagnosis v 55% of patients without metastases at diagnosis; P < .0001). For the group with metastases, there was a trend for better survival for those with lung involvement compared with those with bone metastases or a combination of lung and bone metastases (P < .0001). In the group of patients with no metastases at diagnosis, multivariate analysis demonstrated that site (axial v other), age-group (< 15 v ≥ 15 years), and period of diagnosis had significant influence on RFS (all P < .005). RFS was superior in the period after 1985 compared with the period before 1985 for nonmetastatic patients (45% v 60%, respectively; P < .0001) and for metastatic patients (16% v 30%, respectively; P = .016). Patients who relapsed within 2 years of diagnosis had a less favorable prognosis than patients who relapsed later (5-year survival after relapse, 4% v 23%, respectively; P < .0001). There were other changes over the period; in particular, radiotherapy or amputation were more common in the period before 1986, whereas endoprosthetic surgery was widely used in the later period. CONCLUSION: Survival and RFS improved over the period. Prognostic factors are metastases at diagnosis, primary site, and age.


2012 ◽  
Vol 42 (5) ◽  
pp. 420-426 ◽  
Author(s):  
D. Tural ◽  
N. Molinas Mandel ◽  
S. Dervisoglu ◽  
F. Oner Dincbas ◽  
S. Koca ◽  
...  

2020 ◽  
Author(s):  
Jie Jiang ◽  
Chong Liu ◽  
Guoyong Xu ◽  
Tuo Liang ◽  
Chaojie Yu ◽  
...  

Abstract Background Ewing's sarcoma (ES) is the second most prevalent malignancy among bone tissue tumors, and there is no adequate prognosis biomarker. The protein encoded by CCT6A is a molecular chaperone. Early studies have suggested that CCT6A is involved in the development of many cancers, however, there is no clear evidence of a role for CCT6A in ES. Methods In this study, we performed a bioinformatics analysis of 32 Ewing sarcoma specimens from the GSE17618 dataset for differences in gene expression and overall survival, event-free survival, and gene expression in different subgroups. Results After three screenings, we identified CCT6A as highly correlated with Ewing's sarcoma prognosis. Survival analysis showed low overall survival (OS) for CCT6A high expression (P = 0.024). On the other hand, Cox regression analysis showed that CCT6A expression, event-free survival (EFS), and age were strongly associated with the prognosis of Ewing sarcoma, identified as independent poor prognostic biomarkers. (CCT6A: P = 0.015; Age: P-value = 0.026; EFS: P-value = 0.001). Conclusion The expression level of CCT6A is strongly associated with the prognosis of Ewing's sarcoma. High expression of the CCT6A gene may serve as a biomarker for poor prognosis in patients with Ewing's sarcoma.


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