胸腺上皮肿瘤（TET）是发生于胸腺的罕见恶性肿瘤。尽管如此，TET，包括胸腺瘤（TM）、胸腺癌（TC）和胸腺神经内分泌肿瘤（TNEN），总体上是最常见的纵隔恶性肿瘤。对 TET 进行适当的诊断和治疗管理需要采用多学科方法。迄今为止，主要的治疗策略在很大程度上取决于肿瘤的分期，包括手术联合或不联合新辅助或辅助治疗，以铂类化疗、放疗或放化疗为代表。尽管存在极低的肿瘤突变负荷（TMB）和 TET 中免疫相关不良事件发生率高的挑战，但免疫检查点抑制剂（ICIs）仍在晚期或转移性疾病中进行评估。在这方面，目前正在评估组织生物标志物表达的预测影响，例如程序性细胞死亡配体-1 (PD-L1) 和其他新兴生物标志物，以及它们的最佳和共享解释，以预测 ICI 的反应率。 TETs.© 作者 2024。

Thymic epithelial tumors (TETs) are rare malignant neoplasms arising in the thymus gland. Nevertheless, TETs, including thymomas (TMs), thymic carcinomas (TCs), and thymic neuroendocrine neoplasms (TNENs), are the most common mediastinal malignancies overall. A multidisciplinary approach is required for the appropriate diagnostic and therapeutic management of TETs. To date, the main therapeutic strategies are largely depended on the stage of the tumor and they include surgery with or without neoadjuvant or adjuvant therapy, represented by platinum-based chemotherapy, radiotherapy or chemoradiotherapy. Immune checkpoint inhibitors (ICIs) are ongoing under evaluation in the advanced or metastatic diseases despite the challenges related to the very low tumor mutation burden (TMB) and the high incidence of immune-related adverse events in TETs. In this regard, predictive impact of tissue biomarkers expression such as programmed cell death ligand-1 (PD-L1), and other emerging biomarkers, as well as their optimal and shared interpretation are currently under evaluation in order to predict response rates to ICIs in TETs.© The Author(s) 2024.