胸腺上皮肿瘤（TETs）是起源于胸腺的一类罕见恶性肿瘤。对于早期疾病的患者，手术仍然是治疗的基础。对于不能手术切除、存在转移或复发的TETs，有限的治疗选择与适度的临床疗效相关。免疫治疗在实体瘤治疗中的应用已引起了对其在TET治疗中作用的重视。然而，由于伴随肿瘤的自身免疫性疾病，特别是胸腺瘤，高发的情况，对基于免疫的治疗方法的期望受到了制约。在胸腺瘤和胸腺癌的免疫检查点阻断（ICB）的临床研究中，存在更高的免疫相关不良事件（IRAEs）和有限的疗效。尽管存在这些挫折，对胸腺肿瘤微环境和系统免疫系统的不断深入了解推动了对这些疾病的理解，并提供了新型免疫调节治疗的机会。目前正在进行的研究评估了对TETs的多种基于免疫的治疗，以改善临床疗效并减轻IRAE的风险。本综述将提供对胸腺免疫微环境的现有认识、以往ICB研究的结果以及当前正在探索TET管理的治疗方法的见解。©2023 The Authors。Cancer由Wiley Periodicals LLC代表美国癌症协会出版。

Thymic epithelial tumors (TETs) are a rare group of malignancies arising from the thymus. Surgery remains the foundation of treatment for patients with early-stage disease. Limited treatment options are available for the treatment of unresectable, metastatic, or recurrent TETs and are associated with modest clinical efficacy. The emergence of immunotherapies in the treatment of solid tumors has generated significant interest in understanding their role in TET treatment. However, the high rates of comorbid paraneoplastic autoimmune disorders, particularly in thymoma, have tempered expectations regarding the role of immune-based therapies. Clinical studies of immune checkpoint blockade (ICB) in thymoma and thymic carcinoma have revealed higher frequencies of immune-related adverse events (IRAEs) and limited efficacy. Despite these setbacks, the growing understanding of the thymic tumor microenvironment and systemic immune system has advanced the understanding of these diseases and provided opportunities for novel immunotherapy modalities. Ongoing studies are evaluating numerous immune-based treatments in TETs with the goal of improving clinical efficacy and mitigating IRAE risk. This review will provide insight into the current understanding of the thymic immune microenvironment, outcomes of previous ICB studies, and review treatments currently being explored for the management of TET.© 2023 The Authors. Cancer published by Wiley Periodicals LLC on behalf of American Cancer Society.