scholarly journals Outcomes of Pediatric Patients with metastatic Ewing sarcoma treated with interval compression

2021 ◽  
Author(s):  
Dua'a Zandaki ◽  
Taleb Ismael ◽  
Hadeel Halalsheh ◽  
Ahmad Ibrahimi ◽  
Nasim Sarhan ◽  
...  
Keyword(s):  
Local Control ◽  
Ewing Sarcoma ◽  
Pediatric Patients ◽  
Pulmonary Metastases ◽  
Event Free Survival ◽  
Primary Tumor Site ◽  
Free Survival ◽  
Control Therapy ◽  
Extra Pulmonary ◽  
Metastasis Site

Background: Interval compression (IC), defined as 2 week-long cycles of alternating vincristine/doxorubicin/cyclophosphamide and ifosfamide/etoposide, improves survival for localized Ewing sarcoma. The outcomes of patients with metastatic disease treated with IC are uncertain. Methods: We retrospectively reviewed the charts of pediatric patients with metastatic Ewing sarcoma treated with IC at our center between January-2013 and March-2020. We calculated event-free survival and overall survival and used log rank tests for univariate comparisons. Results: We identified 34 patients aged 2.7–17.1 years (median,11.6 years). Twenty-six patients (76%) had pulmonary metastases, and 14 (41%) had extra-pulmonary metastases in the bone (n = 11), lymph nodes (n = 2), and intraspinal tissue (n = 1). All patients received local control therapy: surgery only (n = 7, 21%), radiotherapy only (n = 18, 53%), or both (n = 9, 26%). The estimated 3-year OS and EFS were 62%±9% and 39%±9%, respectively. Patients with pulmonary only metastasis had a 3-year OS of 88%±8% in comparison to those with extra-pulmonary metastasis of 27%±13% (P=0.0074). Survival did not differ according to age group (> vs < 12 years), metastasis site, or primary tumor site, but 3-year event-free survival significantly differed according to local control therapy (surgery only, 83% ± 15%; combined surgery and radiation, 30% ± 18%; radiation only, 15% ± 10%; P = .048). Conclusion: IC yielded similar outcomes for patients with metastatic Ewing sarcoma to that reported in the literature using other regimens. We suggest including this approach to other blocks of therapy

scholarly journals Primary and Metastatic Intracranial Ewing Sarcoma at Diagnosis: Retrospective International Study and Systematic Review

Cancers ◽  
2020 ◽  
Vol 12 (6) ◽  
pp. 1675
Author(s):  
Lianne M. Haveman ◽  
Andreas Ranft ◽  
Henk van den Berg ◽  
Stephanie Klco-Brosius ◽  
Ruth Ladenstein ◽  
...  
Keyword(s):  
Local Control ◽  
Ewing Sarcoma ◽  
Univariate Analysis ◽  
International Study ◽  
Cerebral Metastases ◽  
The Body ◽  
Event Free Survival ◽  
Free Survival ◽  
Metastatic Lesions ◽  
Chemotherapeutic Treatment

Intracranial Ewing sarcoma (EwS) is rare and publications on primary or metastatic intracranial EwS are minimal. The aim of this study was to describe incidence, clinical behavior, treatment, and factors associated with outcome in patients with primary intracranial EwS or patients with a primary extracranial EwS and cerebral metastases at diagnosis. We reviewed all patients with primary or with metastatic intracranial EwS at diagnosis registered in the International Clinical Trial Euro-E.W.I.N.G.99 (EE99). In total, 17 of 1435 patients (1.2%) presented with primary intracranial EwS; 3 of them had metastatic disease. Four patients (0.3%) with primary extracranial EwS presented with intracranial metastatic lesions. The 3-year event-free survival (EFS) was 64% and overall survival (OS) was 70% in patients with a primary intracranial EwS. Local control in patients with primary intracranial EwS consisted of surgery (6%), radiotherapy (RT) (18%), or both modalities (76%). Univariate analysis showed that patients < 15 years of age had significantly better outcome (EFS: 72%; OS: 76%) compared to those aged above 15 years (EFS: 13%; OS: 25%). In conclusion, primary intracranial EwS and extracranial EwS with cerebral metastases at diagnosis is rare, yet survival is comparable with local and metastatic EwS elsewhere in the body. Age and stage of disease are important prognostic factors. Besides chemotherapeutic treatment, local control with surgical resection combined with RT is recommended whenever feasible.

scholarly journals Adult Ewing Sarcoma: Survival and Local Control Outcomes in 102 Patients with Localized Disease

Sarcoma ◽  
2013 ◽  
Vol 2013 ◽  
pp. 1-7 ◽  
Author(s):  
Safia K. Ahmed ◽  
Steven I. Robinson ◽  
Scott H. Okuno ◽  
Peter S. Rose ◽  
Nadia N. Issa Laack
Keyword(s):  
Local Control ◽  
Ewing Sarcoma ◽  
Poor Prognosis ◽  
Event Free Survival ◽  
Treatment Intensification ◽  
Combined Surgery ◽  
Free Survival ◽  
Entire Cohort ◽  
Improved Outcomes ◽  
Localized Disease

Objectives. To assess the clinical features and local control (LC) outcomes in adult patients with localized Ewing Sarcoma (ES).Methods. The records of 102 ES patients with localized disease ≥18 years of age seen from 1977 to 2007 were reviewed. Factors relevant to prognosis, survival, and LC were analyzed.Results.The 5-year overall survival (OS) and event-free survival (EFS) were 60% and 52%, respectively, for the entire cohort. Treatment era (1977–1992 versus 1993–2007) remained an independent prognostic factor for OS on multivariate analysis, with improved outcomes observed in the 1993–2007 era (P=0.02). The 5-year OS and EFS for the 1993–2007 era were 73% and 60%, respectively. Ifosfamide and etoposide based chemotherapy and surgery were more routinely used in the 1993–2007 era (P<0.01). The 5-year local failure rate (LFR) was 14%, with a 5-year LFR of 18% for surgery, 33% for radiation, and 0% for combined surgery and radiation in the 1993–2007 era (P=0.17).Conclusion. Modern survival outcomes for adults with localized ES are similar to multi-institutional results in children. This improvement over time is associated with treatment intensification with chemotherapy and increased use of surgery. Aggressive LC (combined surgery and radiation) may improve outcomes in poor prognosis patients.

scholarly journals Resection of pulmonary metastases in pediatric patients with Ewing sarcoma improves survival

2011 ◽  
Vol 46 (2) ◽  
pp. 332-335 ◽  
Author(s):  
Phillip A. Letourneau ◽  
Brett Shackett ◽  
Lianchun Xiao ◽  
Jonathan Trent ◽  
Kuo Jen Tsao ◽  
...  
Keyword(s):  
Ewing Sarcoma ◽  
Pediatric Patients ◽  
Pulmonary Metastases

scholarly journals Clinical Impact of Additional Cytogenetic Aberrations, cKIT and RAS Mutations, and Treatment Elements in Pediatric t(8;21)-AML: Results From an International Retrospective Study by the International Berlin-Frankfurt-Münster Study Group

2015 ◽  
Vol 33 (36) ◽  
pp. 4247-4258 ◽  
Author(s):  
Kim Klein ◽  
Gertjan Kaspers ◽  
Christine J. Harrison ◽  
H. Berna Beverloo ◽  
Ardine Reedijk ◽  
...  
Keyword(s):  
Overall Survival ◽  
Complete Remission ◽  
Pediatric Patients ◽  
Lower Probability ◽  
Event Free Survival ◽  
End Points ◽  
Free Survival ◽  
Ras Mutations ◽  
Cytogenetic Aberrations ◽  
High Doses

Purpose This retrospective cohort study aimed to determine the predictive relevance of clinical characteristics, additional cytogenetic aberrations, and cKIT and RAS mutations, as well as to evaluate whether specific treatment elements were associated with outcomes in pediatric t(8;21)-positive patients with acute myeloid leukemia (AML). Patients and Methods Karyotypes of 916 pediatric patients with t(8;21)-AML were reviewed for the presence of additional cytogenetic aberrations, and 228 samples were screened for presence of cKIT and RAS mutations. Multivariable regression models were used to assess the relevance of anthracyclines, cytarabine, and etoposide during induction and overall treatment. End points were the probability of achieving complete remission, cumulative incidence of relapse (CIR), probability of event-free survival, and probability of overall survival. Results Of 838 patients included in final analyses, 92% achieved complete remission. The 5-year overall survival, event-free survival, and CIR were 74%, 58%, and 26%, respectively. cKIT mutations and RAS mutations were not significantly associated with outcome. Patients with deletions of chromosome arm 9q [del(9q); n = 104] had a lower probability of complete remission (P = .01). Gain of chromosome 4 (+4; n = 21) was associated with inferior CIR and survival (P < .01). Anthracycline doses greater than 150 mg/m2 and etoposide doses greater than 500 mg/m2 in the first induction course and high-dose cytarabine 3 g/m2 during induction were associated with better outcomes on various end points. Cumulative doses of cytarabine greater than 30 g/m2 and etoposide greater than 1,500 mg/m2 were associated with lower CIR rates and better probability of event-free survival. Conclusion Pediatric patients with t(8;21)-AML and additional del(9q) or additional +4 might not be considered at good risk. Patients with t(8;21)-AML likely benefit from protocols that have high doses of anthracyclines, etoposide, and cytarabine during induction, as well as from protocols comprising cumulative high doses of cytarabine and etoposide.

Treatment of Pulmonary Metastases in Children With Stage IV Nephroblastoma With Risk-Based Use of Pulmonary Radiotherapy

2012 ◽  
Vol 30 (28) ◽  
pp. 3533-3539 ◽  
Author(s):  
Arnauld Verschuur ◽  
Harm Van Tinteren ◽  
Norbert Graf ◽  
Christophe Bergeron ◽  
Bengt Sandstedt ◽  
...  
Keyword(s):  
Overall Survival ◽  
Survival Rate ◽  
High Risk ◽  
Pulmonary Metastases ◽  
Stage Iv ◽  
Line Treatment ◽  
Event Free Survival ◽  
First Line ◽  
Free Survival ◽  
First Line Treatment

Purpose The purpose of this study was to determine the outcome of children with nephroblastoma and pulmonary metastases (PM) treated according to International Society of Pediatric Oncology (SIOP) 93-01 recommendations using pulmonary radiotherapy (RT) in selected patients. Patients and Methods Patients (6 months to 18 years) were treated with preoperative chemotherapy consisting of 6 weeks of vincristine, dactinomycin, and epirubicin or doxorubicin. If pulmonary complete remission (CR) was not obtained, metastasectomy was considered. Patients in CR received three-drug postoperative chemotherapy, whereas patients not in CR were switched to a high-risk (HR) regimen with an assessment at week 11. If CR was not obtained, pulmonary RT was mandatory. Results Two hundred thirty-four of 1,770 patients had PM. Patients with PM were older (P < .001) and had larger tumor volumes compared with nonmetastatic patients (P < .001). Eighty-four percent of patients were in CR postoperatively, with 17% requiring metastasectomy. Thirty-five patients (16%) had multiple inoperable PM and required the HR protocol. Only 14% of patients received pulmonary RT during first-line treatment. For patients with PM, 5-year event-free survival rate was 73% (95% CI, 68% to 79%), and 5-year overall survival (OS) rate was 82% (95% CI, 77% to 88%). Five-year OS was similar for patients with local stage I and II disease (92% and 90%, respectively) but lower for patients with local stage III disease (68%; P < .001). Patients in CR after chemotherapy only and patients in CR after chemotherapy and metastasectomy had a better outcome than patients with multiple unresectable PM (5-year OS, 88%, 92%, and 48%, respectively; P < .001). Conclusion Following the SIOP protocol, pulmonary RT can be omitted for a majority of patients with PM and results in a relatively good outcome.

Whole lung irradiation for completely responding pulmonary metastases in pediatric Ewing sarcoma

2020 ◽  
Vol 16 (15) ◽  
pp. 1043-1051
Author(s):  
Hagar Elghazawy ◽  
Azza Nasr ◽  
Iman Zaky ◽  
Manal Zamzam ◽  
Ahmed Elgammal ◽  
...  
Keyword(s):  
Pulmonary Metastases ◽  
Pediatric Population ◽  
Event Free Survival ◽  
Relapse Free Survival ◽  
Free Survival ◽  
Real Value ◽  
Lung Irradiation ◽  
Whole Lung Irradiation ◽  
The Impact

Aim: Scarce data assessing the real value of whole lung irradiation (WLI) in Ewing’s sarcoma (ES) with lung-only metastasis, with published conflicting results. We studied the impact of WLI in a homogenous pediatric population. Materials & methods: Retrospective study evaluating the survival outcomes of WLI in these patients. Results: Out of 163 metastatic ES; 41 patients were eligible for WLI. 30 patients (73.1%) received WLI (+ve) while 11 patients (26.8%) did not receive WLI (-ve). Five-year event-free survival was statistically significant in WLI (+ve). Five-year pulmonary relapse-free survival showed trend for improvement with WLI (+ve), while 5-year overall survival was not statistically significant between the two arms. Conclusion: WLI added significantly to the long term clinical outcome of metastatic ES patients, with no irreversible toxicity.

scholarly journals Prognostic Factors for Event-Free Survival in Pediatric Patients with Hepatoblastoma Based on the 2017 PRETEXT and CHIC-HS Systems

Cancers ◽  
2019 ◽  
Vol 11 (9) ◽  
pp. 1387 ◽  
Author(s):  
Hee Mang Yoon ◽  
Jisun Hwang ◽  
Kyung Won Kim ◽  
Jung-Man Namgoong ◽  
Dae Yeon Kim ◽  
...  
Keyword(s):  
Pediatric Patients ◽  
Proportional Hazards ◽  
Hazard Analysis ◽  
Staging System ◽  
Cox Proportional Hazards ◽  
Event Free Survival ◽  
Free Survival ◽  
Tertiary Referral Center ◽  
C Statistic ◽  
Pre Treatment

This study aimed to evaluate the prognostic value of variables used in the 2017 PRE-Treatment EXTent of tumor (PRETEXT) system and the Children’s Hepatic tumors International Collaboration-Hepatoblastoma Stratification (CHIC-HS) system in pediatric patients with hepatoblastoma. A retrospective analysis of data from the pediatric hepatoblastoma registry of a tertiary referral center was conducted to evaluate the clinical and imaging variables (annotation factors) of the PRETEXT staging system. The primary outcome was event-free survival (EFS). Data from 84 patients (mean age: 2.9 ± 3.5 years) identified between 1998 and 2017 were included. Univariable Cox proportional hazards analysis revealed that PRETEXT annotation factors P (portal vein involvement), F (multifocality of tumor), and M (distant metastasis) showed a significant negative association with EFS. Multivariable Cox proportional hazard analysis showed that factor F was the strongest predictor (HR (hazard ratio), 2.908; 95% CI (confidence interval), 1.061–7.972; p = 0.038), whereas factor M showed borderline significance (HR, 2.416; 95% CI, 0.918–6.354; p = 0.074). The prediction model based on F and M (F + M) showed good performance to predict EFS (C-statistic, 0.734; 95% CI, 0.612–0.854). In conclusion, the PRETEXT annotation factor F was the strongest predictor of EFS, and the F + M model showed good performance to predict EFS in pediatric patients with hepatoblastoma.

Role of Surgery in the Treatment of Patients With Stage 4 Neuroblastoma Age 18 Months or Older at Diagnosis

2013 ◽  
Vol 31 (6) ◽  
pp. 752-758 ◽  
Author(s):  
Thorsten Simon ◽  
Beate Häberle ◽  
Barbara Hero ◽  
Dietrich von Schweinitz ◽  
Frank Berthold
Keyword(s):  
Local Control ◽  
Primary Tumor ◽  
Tumor Resection ◽  
Local Control Rate ◽  
Complete Resection ◽  
Incomplete Resection ◽  
Primary Tumor Site ◽  
Free Survival ◽  
Stage 4 ◽  
The Impact

Purpose Although intensive multimodal treatment has improved the prognosis of patients with metastatic neuroblastoma, the impact of primary tumor resection on outcome is a matter of medical debate. Patients and Methods Patients from the German prospective clinical trial NB97 with stage 4 neuroblastoma and age 18 months or older at diagnosis were included. Operation notes and imaging reports were reviewed by two independent experienced physicians. Finally, the extent of tumor resections was correlated with local control rate and outcome. Results A total of 278 patients were included in this study. Image-defined risk factors present at diagnosis were found to be predictive for the extent of tumor resection at first (P < .001) and best (P < .001) operation. No patient died from surgery. Before chemotherapy, complete resection, incomplete resection, and biopsy or no surgery were performed in 6.1%, 5.0%, and 88.5% of patients, respectively. The extent of first operation had no impact on event-free survival (EFS; P = .207), local progression–free survival (LPFS; P = .195), and overall survival (OS; P = .351). After induction chemotherapy, 54.7% of patients underwent complete resection of the primary tumor, 30.6% underwent incomplete resection, and 13.3% had only biopsy or no surgery of the primary tumor. The extent of best operation also had no impact on EFS (P = .877), LPFS (P = .299), and OS (P = .778). Moreover, multivariate analyses showed that surgery did not affect EFS, LPFS, and OS. Conclusion In intensively treated patients with stage 4 neuroblastoma age 18 months or older at diagnosis, surgery of the primary tumor site has no impact on local control rate and outcome.

scholarly journals CCT6A, a Novel Prognostic Biomarker for Ewing's Sarcoma

2020 ◽  
Author(s):  
Jie Jiang ◽  
Chong Liu ◽  
Guoyong Xu ◽  
Tuo Liang ◽  
Chaojie Yu ◽  
...  
Keyword(s):  
Gene Expression ◽  
Overall Survival ◽  
Ewing Sarcoma ◽  
Ewing’S Sarcoma ◽  
Ewing's Sarcoma ◽  
High Expression ◽  
P Value ◽  
Event Free Survival ◽  
Free Survival ◽  
Cox Regression Analysis

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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