Emanuela Palmerini MD, PhD, Peter Reichardt MD, Kirsten Sundby Hall MD, Rossella Bertulli MD, Stefan S. Bielack MD, Alessandro Comandone MD, Gerlinde Egerer MD, Anna Hansmeier MD, Matthias Kevric MD, Elisa Carretta MD, Lina Hansson MD, Nina Jebsen MD, Mikael Eriksson MD, Øyvind S. Bruland MD, Davide Maria Donati MD, PhD, Toni Ibrahim MD, PhD, Sigbjørn Smeland MD, Stefano Ferrari MD
{"title":"多模式治疗罕见原发性恶性骨肉瘤的疗效：来自EUROpean bone Over 40肉瘤研究（EURO-B.O.S.S.）的结果。","authors":"Emanuela Palmerini MD, PhD, Peter Reichardt MD, Kirsten Sundby Hall MD, Rossella Bertulli MD, Stefan S. Bielack MD, Alessandro Comandone MD, Gerlinde Egerer MD, Anna Hansmeier MD, Matthias Kevric MD, Elisa Carretta MD, Lina Hansson MD, Nina Jebsen MD, Mikael Eriksson MD, Øyvind S. Bruland MD, Davide Maria Donati MD, PhD, Toni Ibrahim MD, PhD, Sigbjørn Smeland MD, Stefano Ferrari MD","doi":"10.1002/cncr.34964","DOIUrl":null,"url":null,"abstract":"<div>\n \n \n <section>\n \n <h3> Background</h3>\n \n <p>Rare primary malignant bone sarcomas (RPMBS) account for 5%–10% of primary high-grade bone tumors and represent a major treatment challenge. The outcome of patients with RPMBS enrolled in the <i>EUROpean Bone Over 40 Sarcoma Study</i> (EURO-B.O.S.S) is presented.</p>\n </section>\n \n <section>\n \n <h3> Methods</h3>\n \n <p>Inclusion criteria were as follows: age from 41 to 65 years and a diagnosis of high-grade spindle cell, pleomorphic, or vascular RPMBS. The chemotherapy regimen included doxorubicin 60 mg/m<sup>2</sup>, ifosfamide 9 g/m<sup>2</sup>, and cisplatin 90 mg/m<sup>2</sup>; postoperative methotrexate 8 g/m<sup>2</sup> was added in case of a poor histologic response. Version 2.0 of the Common Terminology Criteria for Adverse Events, Kaplan–Meier curves, log-rank tests, and univariate Cox regression models were used.</p>\n </section>\n \n <section>\n \n <h3> Results</h3>\n \n <p>In total, 113 patients were evaluable for analysis. The median patient age was 52 years (range, 40–66 years), and 67 patients were men. Eighty-eight tumors were categorized as undifferentiated pleomorphic sarcomas (UPS), 20 were categorized as leiomyosarcomas, three were categorized as fibrosarcomas, and two were categorized as angiosarcomas. Eighty-three of 113 tumors were located in the extremities. Ninety-five of 113 patients presented with no evidence of metastases. After a median follow-up of 6.8 years (interquartile range [IQR], 3.5–9.8 years), the 5-year overall survival rate for patients with localized disease was 68.4% (IQR, 56.9%–77.5%), and it was 71.7% (IQR, 58.1%–81.6%) for patients with UPS and 54.9% (IQR, 29.5%–74.5%) for patients with leiomyosarcoma. Grade III–IV hematologic toxicity was reported in 81% patients; 23% had grade II–III neurotoxicity, and 37.5% had grade I–II nephrotoxicity. Five-year overall survival was significantly better for patients with localized disease, for patients who obtained surgical complete remission, and when the primary tumor was located in the extremities.</p>\n </section>\n \n <section>\n \n <h3> Conclusions</h3>\n \n <p>The survival of patients who had RPMBS in the current series was similar to that of age-matched patients who had high-grade osteosarcoma treated according to the same protocol. An osteosarcoma-like chemotherapy may be proposed in patients who have RPMBS.</p>\n </section>\n </div>","PeriodicalId":138,"journal":{"name":"Cancer","volume":"129 22","pages":"3564-3573"},"PeriodicalIF":6.1000,"publicationDate":"2023-08-02","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":"1","resultStr":"{\"title\":\"Outcome of rare primary malignant bone sarcoma treated with multimodal therapy: Results from the EUROpean Bone Over 40 Sarcoma Study (EURO-B.O.S.S.)\",\"authors\":\"Emanuela Palmerini MD, PhD, Peter Reichardt MD, Kirsten Sundby Hall MD, Rossella Bertulli MD, Stefan S. Bielack MD, Alessandro Comandone MD, Gerlinde Egerer MD, Anna Hansmeier MD, Matthias Kevric MD, Elisa Carretta MD, Lina Hansson MD, Nina Jebsen MD, Mikael Eriksson MD, Øyvind S. 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An osteosarcoma-like chemotherapy may be proposed in patients who have RPMBS.</p>\\n </section>\\n </div>\",\"PeriodicalId\":138,\"journal\":{\"name\":\"Cancer\",\"volume\":\"129 22\",\"pages\":\"3564-3573\"},\"PeriodicalIF\":6.1000,\"publicationDate\":\"2023-08-02\",\"publicationTypes\":\"Journal Article\",\"fieldsOfStudy\":null,\"isOpenAccess\":false,\"openAccessPdf\":\"\",\"citationCount\":\"1\",\"resultStr\":null,\"platform\":\"Semanticscholar\",\"paperid\":null,\"PeriodicalName\":\"Cancer\",\"FirstCategoryId\":\"3\",\"ListUrlMain\":\"https://onlinelibrary.wiley.com/doi/10.1002/cncr.34964\",\"RegionNum\":2,\"RegionCategory\":\"医学\",\"ArticlePicture\":[],\"TitleCN\":null,\"AbstractTextCN\":null,\"PMCID\":null,\"EPubDate\":\"\",\"PubModel\":\"\",\"JCR\":\"Q1\",\"JCRName\":\"ONCOLOGY\",\"Score\":null,\"Total\":0}","platform":"Semanticscholar","paperid":null,"PeriodicalName":"Cancer","FirstCategoryId":"3","ListUrlMain":"https://onlinelibrary.wiley.com/doi/10.1002/cncr.34964","RegionNum":2,"RegionCategory":"医学","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":null,"EPubDate":"","PubModel":"","JCR":"Q1","JCRName":"ONCOLOGY","Score":null,"Total":0}
引用次数: 1
摘要
背景：罕见的原发性恶性骨肉瘤（RPMBS）占原发性高级别骨肿瘤的5%-10%，是一个主要的治疗挑战。介绍了参与EUROpean Bone Over 40 Sarcoma研究（EURO-B.O.S.S）的RPMBS患者的结果。方法：纳入标准如下：年龄41至65岁，诊断为高级别梭形细胞、多形性或血管性RPMBS。化疗方案包括阿霉素60mg/m2、异环磷酰胺9g/m2和顺铂90mg/m2；在组织学反应差的情况下，术后加入8 g/m2的甲氨蝶呤。使用2.0版不良事件通用术语标准、Kaplan-Meier曲线、对数秩检验和单变量Cox回归模型。结果：总共有113名患者可进行分析。患者的中位年龄为52岁（40-66岁），67名患者为男性。88例肿瘤被归类为未分化多形性肉瘤（UPS），20例被归类为平滑肌肉瘤，3例被归类于纤维肉瘤，2例被归类至血管肉瘤。113个肿瘤中有83个位于四肢。113例患者中有95例无转移迹象。中位随访6.8年（四分位间距[IQR]，3.5-9.8年）后，局限性疾病患者的5年总生存率为68.4%（IQR，56.9%-77.5%），UPS患者为71.7%（IQR为58.1%-8.16%），平滑肌肉瘤患者为54.9%（IQR：29.5%-74.5%）。81%的患者报告了III-IV级血液学毒性；23%具有Ⅱ-Ⅲ级神经毒性，37.5%具有Ⅰ-Ⅱ级肾毒性。局部疾病患者、手术完全缓解的患者以及原发肿瘤位于四肢的患者的五年总生存率显著提高。结论：本系列中RPMBS患者的生存率与根据相同方案治疗的年龄匹配的高级别骨肉瘤患者相似。对于患有RPMBS的患者，可以建议进行骨肉瘤样化疗。
Outcome of rare primary malignant bone sarcoma treated with multimodal therapy: Results from the EUROpean Bone Over 40 Sarcoma Study (EURO-B.O.S.S.)
Background
Rare primary malignant bone sarcomas (RPMBS) account for 5%–10% of primary high-grade bone tumors and represent a major treatment challenge. The outcome of patients with RPMBS enrolled in the EUROpean Bone Over 40 Sarcoma Study (EURO-B.O.S.S) is presented.
Methods
Inclusion criteria were as follows: age from 41 to 65 years and a diagnosis of high-grade spindle cell, pleomorphic, or vascular RPMBS. The chemotherapy regimen included doxorubicin 60 mg/m2, ifosfamide 9 g/m2, and cisplatin 90 mg/m2; postoperative methotrexate 8 g/m2 was added in case of a poor histologic response. Version 2.0 of the Common Terminology Criteria for Adverse Events, Kaplan–Meier curves, log-rank tests, and univariate Cox regression models were used.
Results
In total, 113 patients were evaluable for analysis. The median patient age was 52 years (range, 40–66 years), and 67 patients were men. Eighty-eight tumors were categorized as undifferentiated pleomorphic sarcomas (UPS), 20 were categorized as leiomyosarcomas, three were categorized as fibrosarcomas, and two were categorized as angiosarcomas. Eighty-three of 113 tumors were located in the extremities. Ninety-five of 113 patients presented with no evidence of metastases. After a median follow-up of 6.8 years (interquartile range [IQR], 3.5–9.8 years), the 5-year overall survival rate for patients with localized disease was 68.4% (IQR, 56.9%–77.5%), and it was 71.7% (IQR, 58.1%–81.6%) for patients with UPS and 54.9% (IQR, 29.5%–74.5%) for patients with leiomyosarcoma. Grade III–IV hematologic toxicity was reported in 81% patients; 23% had grade II–III neurotoxicity, and 37.5% had grade I–II nephrotoxicity. Five-year overall survival was significantly better for patients with localized disease, for patients who obtained surgical complete remission, and when the primary tumor was located in the extremities.
Conclusions
The survival of patients who had RPMBS in the current series was similar to that of age-matched patients who had high-grade osteosarcoma treated according to the same protocol. An osteosarcoma-like chemotherapy may be proposed in patients who have RPMBS.
期刊介绍：
The CANCER site is a full-text, electronic implementation of CANCER, an Interdisciplinary International Journal of the American Cancer Society, and CANCER CYTOPATHOLOGY, a Journal of the American Cancer Society.
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