In the last few years, meaningful advances have been made in the knowledge of the biology of Thymic Epithelial Tumors (TETs). Data available suggest that in most cases, the different histological subtypes could be distinct biological entities, characterized by specific molecular aberrations, rather than representing a histological continuum of diseases. Recurrent gene mutations in Thymomas and Thymic Carcinoma have been identified, but we still do not know the exact role played by these mutations in TETs pathogenesis. Relevant new data are now available on the pathogenetic mechanisms underlying the association between TETs and autoimmune diseases that warrant further investigations for the potential therapeutic implications. The progress in knowledge of the molecular pathways involved in TETs pathogenesis, allowed to identify and to test target therapies potentially active in such diseases. Platinum-based chemotherapy remains the standard first line treatment for patients with advanced or metastatic TETs. However, some promising data have been reported on the activity of new target therapies, including anti-angiogenic drugs, Cycline Dependent Kinases and PI3K/mTOR inhibitors, as well as of Immune-checkpoint inhibitors. A number of new drugs and combinations are currently under evaluation. The efficacy of new drugs should be balanced with their toxicity profiles, in such complex patients that seem to be more susceptible to develop drug-related toxicities, in particular with immunotherapies.

中文翻译：

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胸腺上皮肿瘤：从生物学到治疗方法。

在过去的几年中，胸腺上皮肿瘤（TETs）的生物学知识取得了有意义的进展。现有数据表明，在大多数情况下，不同的组织学亚型可能是不同的生物学实体，其特征是特定的分子畸变，而不是代表疾病的组织学连续性。在胸腺瘤和胸腺癌中已经发现了复发性基因突变，但是我们仍然不知道这些突变在TETs发病机理中的确切作用。现在可获得有关TET和自身免疫性疾病之间关联的致病机制的相关新数据，这些数据值得进一步研究其潜在的治疗意义。TETs发病机理中涉及的分子途径的知识进展，允许识别和测试可能在此类疾病中活跃的目标疗法。铂类化疗仍然是晚期TET或转移性TET患者的标准一线治疗方法。然而，已经报道了一些关于新靶标疗法的活性的有希望的数据，包括抗血管生成药物，细胞周期蛋白依赖性激酶和PI3K / mTOR抑制剂以及免疫检查点抑制剂。目前正在评估许多新药及其组合。在如此复杂的患者中，新药的功效应与其毒性特征相平衡，这些患者似乎更容易产生与药物相关的毒性，特别是免疫疗法。但是，已经报道了一些关于新靶标疗法的活性的有希望的数据，包括抗血管生成药物，细胞周期蛋白依赖性激酶和PI3K / mTOR抑制剂以及免疫检查点抑制剂。目前正在评估许多新药及其组合。在如此复杂的患者中，新药的功效应与其毒性特征相平衡，这些患者似乎更容易产生与药物相关的毒性，特别是免疫疗法。但是，已经报道了一些关于新靶标疗法的活性的有希望的数据，包括抗血管生成药物，细胞周期蛋白依赖性激酶和PI3K / mTOR抑制剂以及免疫检查点抑制剂。目前正在评估许多新药及其组合。在如此复杂的患者中，新药的功效应与其毒性特征相平衡，这些患者似乎更容易产生与药物相关的毒性，特别是免疫疗法。