@article{Mediastinum11358,
author = {Satoru Okada and Shunta Ishihara and Tatsuo Furuya and Chiaki Nakazono and Masayoshi Inoue},
title = {Pathobiology of thymic epithelial tumours and the treatment strategy based on immuno-oncological characteristics: a narrative review},
journal = {Mediastinum},
volume = {10},
number = {0},
year = {2026},
keywords = {},
abstract = {Background and Objective: Thymic epithelial tumours (TETs), including thymomas and thymic carcinomas, are relatively rare anterior mediastinal malignancies. Thymomas are well known to be associated with various autoimmune diseases, such as myasthenia gravis (MG), pure red cell aplasia, and hypogammaglobulinemia. The mainstay of treatment for all TETs is complete surgical resection, although recent advances in irradiation and anti-tumour drug therapies are increasingly indicated for advanced or recurrent disease. The pathobiology of TETs was reviewed to consider optimal treatment strategies based on their immuno-oncological characteristics.Methods: A comprehensive literature search was conducted using PubMed, focusing on the biology and treatment of TETs, along with integration of our original research data.Key Content and Findings: Pathobiology is different between thymoma and thymic carcinoma. Incomplete T cell development is observed in thymoma, which may affect patient autoimmunity. Type AB, B1, and B2 thymomas harbour abundant immature T cells, which cannot act as effector T cells in treatment using immune checkpoint inhibitors (ICIs). Neoplastic thymic epithelial cells (TECs) express programmed cell death-ligand 1 (PD-L1) at varying levels, and frequent genetic aberrations are observed according to the World Health Organization (WHO) classification. Germinal centre formation in the surrounding thymus may have a clinical role in thymoma-associated MG. Regarding treatment strategies, radical minimally invasive thymectomy using robotic technology is now available for MG. Effective molecular-targeted and ICI therapies have recently been introduced, and further novel targeted therapies are currently under development. WHO histological subtypes and inflammatory biomarkers are practically useful for determining the treatment strategy.Conclusions: A fundamental understanding of pathobiology and immuno-oncology is crucial for the appropriate management of TETs.},
issn = {2522-6711}, url = {https://med.amegroups.org/article/view/11358}
}