Chondrosarcomas (CSs) consist of a heterogenous group of primary bone cancers arising from malignant cells which produce cartilaginous matrix. As the second most common primary bone cancer, CS are often resistant to systemic chemotherapy due to poor vascularization, slow proliferation, and expression of multidrug-resistant pumps. Immune checkpoint inhibitors have transformed the field of oncology and are now designated as frontline therapy for many solid tumor cancers. Several studies have demonstrated increased expression of programed cell death 1 (PD-1) and PD-L1 in CS tissue in vitro, which has led to the development of multiple clinical trials for immunotherapy in patients with aggressive CS. In this review, we highlight the ongoing investigation into the role for immunotherapy in CS. We also report the case of a 44-year-old female with a history of stage IV primary CS of the right shoulder who underwent radical resection with recurrence and demonstrated a spectacular sustained response to pembrolizumab at our center. Our review highlights the need for further studies investigating the role of immunotherapy in the treatment of aggressive bone sarcomas that are resistant to standard surgical resection, chemotherapy, and radiation treatment.

中文翻译：

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免疫治疗在软骨肉瘤中的作用：病例报告和文献综述。

软骨肉瘤（CS）由一组异质的原发性骨癌组成，这些癌由产生软骨基质的恶性细胞产生。作为第二常见的原发性骨癌，CS 由于血管化不良、增殖缓慢以及多药耐药泵的表达，通常对全身化疗产生耐药性。免疫检查点抑制剂已经改变了肿瘤学领域，现在被指定为许多实体瘤癌症的一线治疗方法。多项研究表明体外 CS 组织中程序性细胞死亡 1 (PD-1) 和 PD-L1 的表达增加，这导致了针对侵袭性 CS 患者进行免疫治疗的多项临床试验的开展。在这篇综述中，我们重点介绍了正在进行的免疫治疗在 CS 中的作用的研究。我们还报告了一名 44 岁女性的病例，她有右肩 IV 期原发性 CS 病史，她接受了根治性切除术后复发，并在我们中心对派姆单抗表现出惊人的持续反应。我们的综述强调需要进一步研究免疫疗法在治疗对标准手术切除、化疗和放射治疗耐药的侵袭性骨肉瘤中的作用。