Osteoncology, Bone and Soft Tissue Sarcomas, and Innovative Therapies, IRCCS Istituto Ortopedico Rizzoli, Bologna, Italy.
Sarcoma Center, Helios Klinikum Berlin-Buch, Berlin, Germany.
Cancer. 2023 Nov 15;129(22):3564-3573. doi: 10.1002/cncr.34964. Epub 2023 Aug 2.
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/m , ifosfamide 9 g/m , and cisplatin 90 mg/m ; postoperative methotrexate 8 g/m 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.
罕见原发性恶性骨肉瘤（RPMBS）占原发性高级骨肿瘤的 5%-10%，是治疗的主要挑战。本文介绍了入组 EUROpean Bone Over 40 Sarcoma Study（EURO-B.O.S.S）的 RPMBS 患者的结局。
纳入标准为：年龄 41-65 岁，高级梭形细胞、多形性或血管 RPMBS 诊断。化疗方案包括多柔比星 60mg/m²，异环磷酰胺 9g/m²，顺铂 90mg/m²；如果组织学反应不佳，术后加用甲氨蝶呤 8g/m²。采用第 2.0 版常见不良事件术语标准、Kaplan-Meier 曲线、对数秩检验和单变量 Cox 回归模型进行分析。
共 113 例患者可评估分析。中位患者年龄为 52 岁（范围 40-66 岁），67 例为男性。88 例肿瘤为未分化多形性肉瘤（UPS），20 例为平滑肌肉瘤，3 例为纤维肉瘤，2 例为血管肉瘤。113 例肿瘤中有 83 例位于四肢。95 例患者无转移证据。中位随访 6.8 年（四分位距[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：58.1%-81.6%），平滑肌肉瘤患者为 54.9%（IQR：29.5%-74.5%）。81%的患者报告有 3-4 级血液学毒性；23%有 2-3 级神经毒性，37.5%有 1-2 级肾毒性。局限性疾病、获得手术完全缓解以及肿瘤位于四肢的患者 5 年总生存率显著提高。
本研究中 RPMBS 患者的生存情况与按相同方案治疗的年龄匹配的高级骨肉瘤患者相似。对于患有 RPMBS 的患者，可提出类似于骨肉瘤的化疗方案。
