Thymic epithelial tumors (TETs), comprising thymoma and thymic carcinoma, are rare mediastinal neoplasms that pose significant diagnostic and therapeutic challenges due to their complex anatomy and frequent association with paraneoplastic syndromes like myasthenia gravis (MG). This narrative review synthesizes recent advances in the multidisciplinary management of TETs, providing clinicians with a contemporary, tumor-specific overview. We conducted systematic literature searches across PubMed, Scopus, and Web of Science for publications from January 2018 to June 2024, focusing on high-impact studies (impact factor >3) with sample sizes >10 patients. Key terms included "thymoma", "thymic carcinoma", "minimally invasive thymectomy", "targeted therapy", and "immunotherapy". Content screening prioritized TETs-specific evidence. Innovations in computed tomography (CT), magnetic resonance imaging (MRI), and positron emission tomography/CT (PET/CT) have enhanced TETs characterization, though differentiation of thymic hyperplasia remains challenging. Surgical management has shifted toward minimally invasive techniques [uniportal video-assisted thoracoscopic surgery (VATS), subxiphoid approach] and robotic surgery, reducing morbidity while maintaining oncological outcomes. For giant or invasive TETs, extended open techniques like clamshell incision remain indispensable. Systemic therapy advances include KIT/PIK3CA-targeted agents (imatinib, everolimus) showing modest efficacy, while programmed death-1/programmed death-ligand 1 (PD-1/PD-L1) inhibitors demonstrate 20-30% response rates in thymic carcinoma but are associated with a >50% risk of severe immune-related adverse events (irAEs). Platinum-based chemotherapy [cisplatin, doxorubicin, cyclophosphamide (CAP) regimen] remains standard for advanced disease. Multidisciplinary team (MDT) integration is fundamental, particularly for managing MG perioperatively. TETs management is evolving rapidly through technological and biological advances. Future progress hinges on: (I) validating artificial intelligence (AI)-driven imaging classifiers; (II) conducting randomized controlled trials (RCTs) comparing surgical approaches; (III) elucidating immunotherapy toxicity mechanisms; and (IV) developing predictive composite biomarkers integrating genomic, immunological, and clinical parameters to enable precision medicine while mitigating fatal toxicities.
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