Abstract
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.
Highlights
Thymic epithelial tumors (TETs) are a group of rare thoracic malignancies including thymic carcinomas (TC) and thymomas (Tm), with an annual incidence rate ranging between 0.9 and 2.3 per million [1]
We reviewed the most recent understanding of TET immunopathology, and existing data on immunotherapy to put into frame the immune-related events in TET patients and provide a focus for ongoing studies and future perspectives in the treatment of this rare group of diseases
Auto Immune Regulator (AIRE) is a transcriptional factor highly expressed in medullary thymic epithelial cells, which plays a pivotal role in T-cell negative selection
Summary
Thymic epithelial tumors (TETs) are a group of rare thoracic malignancies including thymic carcinomas (TC) and thymomas (Tm), with an annual incidence rate ranging between 0.9 and 2.3 per million [1]. TET patients often present with immune-mediated paraneoplastic syndromes. The role of thymus in the development of adaptive immunity and the altered molecular pathways involved in TET might explain the high rate of paraneoplastic syndromes in these patients [13,14,15,16]. Immunotherapy with immune checkpoint inhibitors is being investigated in TETs, yielding interesting response rates and survival outcomes. We reviewed the most recent understanding of TET immunopathology, and existing data on immunotherapy to put into frame the immune-related events in TET patients and provide a focus for ongoing studies and future perspectives in the treatment of this rare group of diseases
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