Thymic epithelial tumors (TETs) comprise a rare group of thoracic cancers, classified as thymomas and thymic carcinomas (TC). To date, chemotherapy is still the standard treatment for advanced disease. Unfortunately, few therapeutic options are available for relapsed/refractory tumors. Unlike other solid cancers, the development of targeted biologic and/or immunologic therapies in TETs remains in its nascent stages. Moreover, since the thymus plays a key role in the development of immune tolerance, thymic tumors have a unique biology, which can confer susceptibility to autoimmune diseases and ultimately influence the risk–benefit balance of immunotherapy, especially for patients with thymoma. Indeed, early results from single-arm studies have shown interesting clinical activity, albeit at a cost of a higher incidence of immune-related side effects. The lack of knowledge of the immune mechanisms associated with TETs and the absence of biomarkers predictive of response or toxicity to immunotherapy risk limiting the evolution of immunotherapeutic strategies for managing these rare tumors. The aim of this review is to summarize the existing literature about the thymus’s immune biology and its association with autoimmune paraneoplastic diseases, as well as the results of the available studies with immune checkpoint inhibitors and cancer vaccines.
胸腺上皮肿瘤（TETs）是一类罕见的胸部恶性肿瘤，主要包括胸腺瘤与胸腺癌。目前，化疗仍是晚期疾病的标准治疗方案，但针对复发/难治性肿瘤的治疗选择极为有限。与其他实体肿瘤不同，针对TETs的靶向生物治疗和/或免疫治疗仍处于起步阶段。由于胸腺在免疫耐受形成过程中发挥关键作用，胸腺肿瘤具有独特的生物学特性，可能导致患者易患自身免疫性疾病，并最终影响免疫治疗的风险-获益平衡，这一特点在胸腺瘤患者中尤为显著。单臂研究的早期数据显示，免疫治疗虽展现出具有前景的临床活性，但同时也伴随着较高的免疫相关不良反应发生率。目前对TETs相关免疫机制认知的不足，以及缺乏能够预测免疫治疗反应或毒性的生物标志物，限制了针对这类罕见肿瘤的免疫治疗策略的发展。本文旨在综述现有文献中关于胸腺免疫生物学特性及其与副肿瘤性自身免疫疾病关联的研究，同时总结免疫检查点抑制剂和癌症疫苗在TETs治疗中的现有研究成果。
Thymic Epithelial Tumor and Immune System: The Role of Immunotherapy
肿瘤(癌症)患者之家