Primary leiomyosarcoma of bone (LMSoB) is extremely rare, comprising only <0.7% of primary malignant bone tumors, and is therefore considered an ultra-rare tumor entity. There is currently no consensus as to whether therapeutic strategies should be based on the biological characteristics of soft tissue leiomyosarcoma or on primary tumor localization in the bone. The use of perioperative chemotherapy and its effectiveness in this rare tumor entity remains unclear. We aimed to evaluate the impact of different treatment approaches in a multicenter setting with a total of 35 patients included. The 5-year overall survival (OS) was 74%. Patients with localized disease undergoing surgery had a significantly higher 5-year OS compared to patients who did not undergo surgical treatment (82% vs. 0%,p= 0.0015). Axial tumor localization was associated with worse event-free survival (EFS) probability (p< 0.001) and OS (p= 0.0082). A high proportion of our patients developed secondary metastases. Furthermore, the perioperative chemotherapy protocols applied to our patients were not associated with an improved EFS or OS. Therefore, the benefit of perioperative chemotherapy in LMSoB needs to be further investigated, and the choice of agents still needs to be clarified.
原发性骨平滑肌肉瘤极为罕见，仅占原发性恶性骨肿瘤的不到0.7%，因此被视为一种超罕见肿瘤类型。目前对于其治疗策略应基于软组织平滑肌肉瘤的生物学特性，还是基于骨内原发肿瘤的定位，尚未达成共识。围手术期化疗的应用及其在这种罕见肿瘤中的疗效仍不明确。本研究旨在多中心环境下评估不同治疗方案对35例患者的影响。五年总生存率为74%。接受手术治疗的局限性病变患者，其五年总生存率显著高于未接受手术治疗的患者（82%对比0%，p=0.0015）。中轴骨肿瘤定位与较差的无事件生存率（p<0.001）和总生存率（p=0.0082）相关。本研究中大部分患者出现了继发性转移。此外，患者接受的围手术期化疗方案与无事件生存率或总生存率的改善无显著关联。因此，围手术期化疗在骨平滑肌肉瘤中的获益仍需进一步研究，其药物选择也有待明确。
肿瘤(癌症)患者之家