Victor Rechl, Andreas Ranft, Vivek Bhadri, Benedicte Brichard, Stephane Collaud, Sona Cyprova, Hans Eich, Torben Ek, Hans Gelderblom, Jendrik Hardes, Lianne M Haveman, Wolfgang Hartmann, Peter Hauser, Philip Heesen, Heribert Jürgens, Jukka Kanerva, Thomas Kühne, Anna Raciborska, Jelena Rascon, Arne Streitbürger, Yasmin Uhlenbruch, Beate Timmermann, Josephine Kersting, Minh Thanh Pham, Uta Dirksen
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We analyzed factors that might influence the outcome of patients suffering from sacral EwS.</p><p><strong>Methods: </strong>We retrospectively analyzed data of the GPOH EURO-E.W.I.N.G 99 trial and the EWING 2008 trial, with a cohort of 124 patients with localized or metastatic sacral EwS. The study endpoints were overall survival (OS) and event-free survival (EFS). OS and EFS were calculated using the Kaplan-Meier method, and univariate comparisons were estimated using the log-rank test. Hazard ratios (HRs) with respective 95% confidence intervals (CIs) were estimated in a multivariable Cox regression model.</p><p><strong>Results: </strong>The presence of metastases (3y-EFS: 0.33 vs. 0.68; <i>P</i> < 0.001; HR = 3.4, 95% CI 1.7 to 6.6; 3y-OS: 0.48 vs. 0.85; <i>P</i> < 0.001; HR = 4.23, 95% CI 1.8 to 9.7), large tumor volume (≥200 ml) (3y-EFS: 0.36 vs. 0.69; <i>P</i>=0.02; HR = 2.1, 95% CI 1.1 to 4.0; 3y-OS: 0.42 vs. 0.73; <i>P</i>=0.04; HR = 2.1, 95% CI 1.03 to 4.5), and age ≥18 years (3y-EFS: 0.41 vs. 0.60; <i>P</i>=0.02; HR = 2.6, 95% CI 1.3 to 5.2; 3y-OS: 0.294 vs. 0.59; <i>P</i>=0.01; HR = 2.92, 95% CI 1.29 to 6.6) were revealed as adverse prognostic factors.</p><p><strong>Conclusion: </strong>Young age seems to positively influence patients` survival, especially in patients with primary metastatic disease. In this context, our results support other studies, stating that older age has a negative impact on survival. Tumor volume, metastases, and the type of local therapy modality have an impact on the outcome of sacral EwS. Level of evidence: Level 2. This trial is registered with NCT00020566 and NCT00987636.</p>","PeriodicalId":21431,"journal":{"name":"Sarcoma","volume":"2024 ","pages":"4751914"},"PeriodicalIF":0.0000,"publicationDate":"2024-03-16","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://www.ncbi.nlm.nih.gov/pmc/articles/PMC10960648/pdf/","citationCount":"0","resultStr":"{\"title\":\"Factors Influencing the Outcome of Patients with Primary Ewing Sarcoma of the Sacrum.\",\"authors\":\"Victor Rechl, Andreas Ranft, Vivek Bhadri, Benedicte Brichard, Stephane Collaud, Sona Cyprova, Hans Eich, Torben Ek, Hans Gelderblom, Jendrik Hardes, Lianne M Haveman, Wolfgang Hartmann, Peter Hauser, Philip Heesen, Heribert Jürgens, Jukka Kanerva, Thomas Kühne, Anna Raciborska, Jelena Rascon, Arne Streitbürger, Yasmin Uhlenbruch, Beate Timmermann, Josephine Kersting, Minh Thanh Pham, Uta Dirksen\",\"doi\":\"10.1155/2024/4751914\",\"DOIUrl\":null,\"url\":null,\"abstract\":\"<p><strong>Background: </strong>Ewing sarcoma (EwS) is a rare and highly malignant bone tumor primarily affecting children, adolescents, and young adults. The pelvis, trunk, and lower extremities are the most common sites, while EwS of the sacrum as a primary site is very rare, and only few studies focusing on this location are published. Due to the anatomical condition, local treatment is challenging in sacral malignancies. We analyzed factors that might influence the outcome of patients suffering from sacral EwS.</p><p><strong>Methods: </strong>We retrospectively analyzed data of the GPOH EURO-E.W.I.N.G 99 trial and the EWING 2008 trial, with a cohort of 124 patients with localized or metastatic sacral EwS. The study endpoints were overall survival (OS) and event-free survival (EFS). OS and EFS were calculated using the Kaplan-Meier method, and univariate comparisons were estimated using the log-rank test. Hazard ratios (HRs) with respective 95% confidence intervals (CIs) were estimated in a multivariable Cox regression model.</p><p><strong>Results: </strong>The presence of metastases (3y-EFS: 0.33 vs. 0.68; <i>P</i> < 0.001; HR = 3.4, 95% CI 1.7 to 6.6; 3y-OS: 0.48 vs. 0.85; <i>P</i> < 0.001; HR = 4.23, 95% CI 1.8 to 9.7), large tumor volume (≥200 ml) (3y-EFS: 0.36 vs. 0.69; <i>P</i>=0.02; HR = 2.1, 95% CI 1.1 to 4.0; 3y-OS: 0.42 vs. 0.73; <i>P</i>=0.04; HR = 2.1, 95% CI 1.03 to 4.5), and age ≥18 years (3y-EFS: 0.41 vs. 0.60; <i>P</i>=0.02; HR = 2.6, 95% CI 1.3 to 5.2; 3y-OS: 0.294 vs. 0.59; <i>P</i>=0.01; HR = 2.92, 95% CI 1.29 to 6.6) were revealed as adverse prognostic factors.</p><p><strong>Conclusion: </strong>Young age seems to positively influence patients` survival, especially in patients with primary metastatic disease. In this context, our results support other studies, stating that older age has a negative impact on survival. Tumor volume, metastases, and the type of local therapy modality have an impact on the outcome of sacral EwS. Level of evidence: Level 2. 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引用次数: 0
摘要
背景:尤文肉瘤(EwS)是一种罕见的高度恶性骨肿瘤,主要影响儿童、青少年和年轻人。骨盆、躯干和下肢是最常见的发病部位,而以骶骨为主要发病部位的尤文氏肉瘤则非常罕见,目前仅有极少数针对该部位的研究发表。由于解剖条件的限制,骶骨恶性肿瘤的局部治疗具有挑战性。我们分析了可能影响骶骨恶性肿瘤患者预后的因素:我们回顾性分析了 GPOH EURO-E.W.I.N.G 99 试验和 EWING 2008 试验的数据,其中包括 124 例局部或转移性骶骨 EwS 患者。研究终点为总生存期(OS)和无事件生存期(EFS)。OS 和 EFS 采用 Kaplan-Meier 法计算,单变量比较采用 log-rank 检验进行估计。在多变量考克斯回归模型中估算了危险比(HRs)及各自的95%置信区间(CIs):出现转移(3y-EFS:0.33 vs. 0.68;P <0.001;HR = 3.4,95% CI 1.7 to 6.6;3y-OS:0.48 vs. 0.85;P <0.001;HR = 4.23,95% CI 1.8 至 9.7)、肿瘤体积大(≥200 ml)(3y-EFS:0.36 vs. 0.69;P=0.02;HR = 2.1,95% CI 1.1 至 4.0;3y-OS:0.42 vs. 0.73; P=0.04; HR = 2.1, 95% CI 1.03 to 4.5)和年龄≥18岁(3y-EFS: 0.41 vs. 0.60; P=0.02; HR = 2.6, 95% CI 1.3 to 5.2; 3y-OS: 0.294 vs. 0.59; P=0.01; HR = 2.92, 95% CI 1.29 to 6.6)被认为是不良预后因素:结论:年轻似乎对患者的生存有积极影响,尤其是对原发转移性疾病患者。在这种情况下,我们的研究结果与其他研究结果一致,即年龄越大,生存率越低。肿瘤体积、转移灶和局部治疗方式对骶骨EwS的预后有影响。证据级别:2级。该试验已在 NCT00020566 和 NCT00987636 上注册。
Factors Influencing the Outcome of Patients with Primary Ewing Sarcoma of the Sacrum.
Background: Ewing sarcoma (EwS) is a rare and highly malignant bone tumor primarily affecting children, adolescents, and young adults. The pelvis, trunk, and lower extremities are the most common sites, while EwS of the sacrum as a primary site is very rare, and only few studies focusing on this location are published. Due to the anatomical condition, local treatment is challenging in sacral malignancies. We analyzed factors that might influence the outcome of patients suffering from sacral EwS.
Methods: We retrospectively analyzed data of the GPOH EURO-E.W.I.N.G 99 trial and the EWING 2008 trial, with a cohort of 124 patients with localized or metastatic sacral EwS. The study endpoints were overall survival (OS) and event-free survival (EFS). OS and EFS were calculated using the Kaplan-Meier method, and univariate comparisons were estimated using the log-rank test. Hazard ratios (HRs) with respective 95% confidence intervals (CIs) were estimated in a multivariable Cox regression model.
Results: The presence of metastases (3y-EFS: 0.33 vs. 0.68; P < 0.001; HR = 3.4, 95% CI 1.7 to 6.6; 3y-OS: 0.48 vs. 0.85; P < 0.001; HR = 4.23, 95% CI 1.8 to 9.7), large tumor volume (≥200 ml) (3y-EFS: 0.36 vs. 0.69; P=0.02; HR = 2.1, 95% CI 1.1 to 4.0; 3y-OS: 0.42 vs. 0.73; P=0.04; HR = 2.1, 95% CI 1.03 to 4.5), and age ≥18 years (3y-EFS: 0.41 vs. 0.60; P=0.02; HR = 2.6, 95% CI 1.3 to 5.2; 3y-OS: 0.294 vs. 0.59; P=0.01; HR = 2.92, 95% CI 1.29 to 6.6) were revealed as adverse prognostic factors.
Conclusion: Young age seems to positively influence patients` survival, especially in patients with primary metastatic disease. In this context, our results support other studies, stating that older age has a negative impact on survival. Tumor volume, metastases, and the type of local therapy modality have an impact on the outcome of sacral EwS. Level of evidence: Level 2. This trial is registered with NCT00020566 and NCT00987636.
SarcomaMedicine-Radiology, Nuclear Medicine and Imaging
CiteScore
5.00
自引率
0.00%
发文量
15
审稿时长
14 weeks
期刊介绍:
Sarcoma is dedicated to publishing papers covering all aspects of connective tissue oncology research. It brings together work from scientists and clinicians carrying out a broad range of research in this field, including the basic sciences, molecular biology and pathology and the clinical sciences of epidemiology, surgery, radiotherapy and chemotherapy. High-quality papers concerning the entire range of bone and soft tissue sarcomas in both adults and children, including Kaposi"s sarcoma, are published as well as preclinical and animal studies. This journal provides a central forum for the description of advances in diagnosis, assessment and treatment of this rarely seen, but often mismanaged, group of patients.