稀有的原发性恶性骨肉瘤(RPMBS)占原发高级别骨肿瘤的5％-10％，对治疗构成重大挑战。我们呈现了参加欧洲40岁以上骨肉瘤研究(EURO-B.O.S.S)的RPMBS患者的结果。纳入标准如下：年龄在41岁到65岁之间，并且被诊断为高级别的纺锤细胞、多形性或血管性的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，术后甲氨蝶呤8g/m2会被添加。版本2.0的不良事件通用术语标准、Kaplan-Meier曲线、log-rank检验和单变量Cox回归模型被使用。总共，有113名患者适合分析。患者中位年龄为52岁(范围为40-66岁)，67名患者为男性。88个肿瘤被归类为未分化多形性肉瘤(UPS)，20个被归类为平滑肌肉瘤，3个被归类为纤维肉瘤，2个被归类为血管肉瘤。113个肿瘤中有83个位于四肢。113名患者中95名无转移表现。在中位随访6.8年(四分位距[1QR]，3.5-9.8年)后，局部疾病患者的5年总生存率为68.4%(IQR，56.9%-77.5%)，而UPS患者为71.7%(IQR，58.1%-81.6%)，平滑肌肉瘤患者为54.9%(IQR，29.5%-74.5%)。81％患者报告了Ⅲ-Ⅳ级造血系统毒性；23％患有Ⅱ-Ⅲ级神经毒性，37.5％患有Ⅰ-Ⅱ级肾毒性。局部疾病患者、获得手术完全缓解的患者以及原发肿瘤位于四肢的患者的5年总生存明显更好。当前系列中RPMBS患者的生存率与按照相同方案进行治疗的年龄相配的高级别骨肉瘤患者相似。在RPMBS患者中可以提议使用类似骨肉瘤的化疗方案。© 2023年癌症文章作者。由Wiley Periodicals LLC代表美国癌症学会发表。

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.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.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.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.© 2023 The Authors. Cancer published by Wiley Periodicals LLC on behalf of American Cancer Society.