Thymic malignancies: role of immunotherapy and novel approaches.

Abstract

Thymic epithelial tumors (TETs) are a diverse group of malignancies that include thymomas (T), thymic carcinomas (TC), and thymic neuroendocrine tumors. Given the rarity of this disease, evidence defining the optimal treatment approach in the advanced/metastatic setting is limited. This article reviews the latest advances in systemic therapy for TETs, with a special focus on immunotherapy and targeted therapy strategies. Multiple recent efforts have been made to integrate novel immunotherapies and targeted therapy approaches into the current treatment algorithm for T and TC. In addition to trials of checkpoint inhibitor monotherapy, combinatorial approaches with novel immunotherapies or targeted therapies are being explored. Molecular profiling may help identify druggable targets, further optimizing outcomes in this population. Immune checkpoint inhibitor therapy has shown promising activity in TETs patients. However, toxicity in an unselected cohort, particularly in T patients, can be substantial, and therefore it is not recommended outside of clinical trials. Until additional research validates biomarkers to safely select patients for immunotherapy, targeted therapies remain a reasonable second-line option. Contemporary next-generation sequencing panels may be applied to identify druggable targets in the absence of standard treatment.