Abstract
Thymomas and thymic carcinomas (TCs) (also known as Thymic Epithelial Tumors or TETs) are rare cancers and the most frequent masses of the anterior mediastinum. These tumors appear in the epithelial component of the thymus, a primary lymphoid organ, and they have reported a high risk of auto-immunity due to a unique biology. Indeed, up to 30% of patients with TETs could present an autoimmune disorder (AID), the most frequent being Myasthenia Gravis (MG). Moreover, AIDs have been reported not only at tumor diagnosis but before and during the follow-up. These tumors have a lack of specific therapeutic targets for metastatic setting. Immune checkpoint inhibitors (ICI) may defeat cancer cells' capacity to evade the immune system and proliferate. The long-term benefit of ICIs in the metastatic setting in several tumors, such as melanoma or non-small cell lung cancer (NSCLC), let to evaluate ICI approaches in TETs. The high rate of AIDs and distribution of autoimmune events among TET's histological subtypes may have an influence on the decision regarding a treatment based on ICI due to the increased risk of toxicity. We summarize the current evidence for the efficacy of ICI in thymoma and TC and discuss several unresolved challenges and concerns for the use of this agents in TETs.