去分化软骨肉瘤诊疗进展  

Progress in the diagnosis and treatment of dedifferentiated chondrosarcoma

作　　者：徐子博 李卓宇 刘巍峰 Xu Zibo;Li Zhuoyu;Liu Weifeng(Department of Orthopaedics Oncology Surgery,Beijing Jishuitan Hospital,Capital Medical University,National Orthopaedic Medical Center,Beijing 100035,China)

机构地区：[1]首都医科大学附属北京积水潭医院骨肿瘤科,国家骨科医学中心,北京100035

出　　处：《中华骨科杂志》2024年第20期1371-1376,共6页Chinese Journal of Orthopaedics

基　　金：国家重点研发计划(2021YFC2400500);国家自然科学基金(52473310);北京市自然科学基金(L212042)。

摘　　要：去分化软骨肉瘤(dedifferentiated chondrosarcoma,DDCS)是一种高级别软骨肉瘤,发病率低,预后极差,诊断和治疗均较为困难。详尽的影像学检查是DDCS诊断的基础,影像引导下的穿刺活检是确诊的重要手段。手术仍然是目前DDCS治疗的主要方式,应当根据患者具体情况选择合适的手术方案。获得良好的外科切缘是防止DDCS局部复发和远处转移的关键。DDCS对放化疗均不敏感,新的放疗技术和化疗方案仍处于临床探索阶段。近年来国内外学者不断尝试将免疫治疗、靶向治疗等新兴治疗手段运用于DDCS的治疗中,并取得了显著的成果。但是由于DDCS较为罕见,现有临床研究多为病例报告或样本量较少的回顾性研究,未来还需进一步研究以提供更有力的临床证据。Dedifferentiated chondrosarcoma(DDCS)is a rare,high-grade variant of chondrosarcoma with a poor prognosis,making its diagnosis and treatment particularly challenging.Comprehensive imaging plays a crucial role in the initial diagnosis,while image-guided biopsy remains an essential tool for confirming the disease.Surgical intervention remains the primary treatment modality,and selecting an appropriate surgical approach based on the patient's condition is critical.Achieving wide or radical surgical margins is vital for reducing the risk of local recurrence and distant metastasis.Unfortunately,DDCS demonstrates limited sensitivity to both radiotherapy and chemotherapy,and current advancements in these therapeutic approaches remain in the exploratory phase.In recent years,efforts by both domestic and international researchers to incorporate novel therapies such as immunotherapy and targeted therapy have yielded promising,results.Given the rarity of DDCS,existing clinical studies are largely limited to case reports or small retrospective studies,highlighting the need for larger,more robust studies to strengthen the evidence base.This review aims to summarize the latest progress in the diagnosis and treatment of DDCS,offering insights to guide clinical practice and inform future research directions.

关 键 词：软骨肉瘤 诊断 外科手术 预后 

分 类 号：R738.3[医药卫生—肿瘤]