Wu Xiaoli, Lin Xiangwu, Chen Ying, Kong Wencui, Xu Jinhe, Yu Zongyang
Fu Zong Clinical Medicine, Fujian Medical University, Fuzhou, Fujian, China.
Department of Medical Oncology, The 900th Hospital of the Joint Logistic Support Force, PLA, Fuzhou, China.
Front Oncol. 2020 Dec 17;10:565945. doi: 10.3389/fonc.2020.565945. eCollection 2020.
Chordoma is a rare primary bone tumor that exhibits insensitivity to radiotherapy and chemotherapy and has a poor prognosis. Currently, resection is the primary treatment for affected patients, but the subsequent rate of recurrence is high, and both overall survival (OS) and progression-free survival (PFS) are consequentially relatively short. This case report describes a patient who was diagnosed with metastatic chordoma that was found to possess the A1209fs mutation of the gene, which may be associated with beneficial responses to immunotherapies. The patient received pembrolizumab, an immune checkpoint inhibitor (ICI) that targets the PD-1 receptor of lymphocytes, as second-line therapy, which he tolerated well (the most frequent adverse events were abnormal liver function and hyperglycemia, both of which were only grades 1-2), and achieved a PFS duration of 9.3 months. We hope these results will promote further research that will clarify the mechanisms underlying this beneficial response and that will further explore the use of immunotherapies in this population.
脊索瘤是一种罕见的原发性骨肿瘤，对放疗和化疗不敏感，预后较差。目前，手术切除是患病患者的主要治疗方法，但随后的复发率很高，因此总生存期（OS）和无进展生存期（PFS）都相对较短。本病例报告描述了一名被诊断为转移性脊索瘤的患者，发现其基因存在A1209fs突变，这可能与对免疫疗法的有益反应相关。该患者接受了派姆单抗（一种靶向淋巴细胞PD-1受体的免疫检查点抑制剂[ICI]）作为二线治疗，他耐受性良好（最常见的不良事件是肝功能异常和高血糖，均为1-2级），并实现了9.3个月的无进展生存期。我们希望这些结果将推动进一步的研究，以阐明这种有益反应的潜在机制，并进一步探索在该人群中使用免疫疗法。
