Department of Experimental, Diagnostic and Specialty Medicine, Policlinico di Sant'Orsola University Hospital, Via P. Albertoni 15, 40138 Bologna, Italy.
Oncologia Medica, Azienda Ospedaliero-Universitaria di Bologna, Via Albertoni 15, 40138 Bologna, Italy.
Int J Mol Sci. 2020 Nov 28;21(23):9056. doi: 10.3390/ijms21239056.
Thymic epithelial tumors (TETs) are a group of rare thoracic malignancies, including thymic carcinomas (TC) and thymomas (Tm). Autoimmune paraneoplastic diseases are often observed in TETs, especially Tms. To date, chemotherapy is still the standard treatment for advanced disease. Unfortunately, few therapeutic options are available for relapsed/refractory TETs. In the last few years, the deepening of knowledge on thymus' immunobiology and involved altered genetic pathways have laid the foundation for new treatment options in these rare neoplasms. Recently, the immunotherapy revolution has landed in TETs, showing both a dark and light side. Indeed, despite the survival benefit, the occurrence of severe autoimmune treatment-related adverse events has risen crescent uncertainty about the feasibility of immunotherapy in these patients, prone to autoimmunity for their cancer biology. In this review, after summarizing immunobiology and immunopathology of TETs, we discuss available data on immune-checkpoint inhibitors and future perspectives of this therapeutic strategy.
胸腺上皮肿瘤（TETs）是一组罕见的胸部恶性肿瘤，包括胸腺癌（TC）和胸腺瘤（Tm）。自身免疫性副肿瘤疾病在 TETs 中经常观察到，特别是在 Tms 中。迄今为止，化疗仍然是晚期疾病的标准治疗方法。不幸的是，对于复发/难治性 TETs 几乎没有治疗选择。在过去的几年中，对胸腺免疫生物学的深入了解和涉及的遗传途径改变为这些罕见肿瘤的新治疗选择奠定了基础。最近，免疫治疗的革命已经在 TETs 中落地，展示了正反两面。事实上，尽管有生存获益，但严重的自身免疫治疗相关不良事件的发生对免疫疗法在这些患者中的可行性增加了越来越多的不确定性，因为他们的癌症生物学容易发生自身免疫。在这篇综述中，我们总结了 TETs 的免疫生物学和免疫病理学，讨论了免疫检查点抑制剂的现有数据和这种治疗策略的未来展望。
