Background and Objective:Thymic epithelial tumors (TETs), including thymoma and thymic carcinoma, are rare tumors. Although systemic therapy is commonly used for unresectable advanced or recurrent TETs, establishing robust evidence remains challenging. This narrative review summarizes current evidence and incorporates the latest clinical trial data, reporting outcomes separately for thymoma and thymic carcinoma whenever possible. We also highlight practical considerations relevant to clinical practice, including dosing strategies and toxicity management. Methods:We searched PubMed and Google Scholar for prospective and retrospective original studies, meta-analyses, review articles, and case studies published up to November 2025 using terms including "thymoma", "thymic carcinoma", "thymic cancer", "thymic tumors", "thymic epithelial tumors", "chemotherapy", "systemic treatment", "immunotherapy", and "targeted therapy". Ongoing trials were identified via ClinicalTrials.gov and the Japan Registry of Clinical Trials. Retrospective studies enrolling fewer than 15 participants were excluded. Key Content and Findings:Systemic therapy for advanced TETs continues to include cytotoxic chemotherapy as a major component. In selected settings, particularly in thymic carcinoma, targeted therapy and immune checkpoint inhibitors (ICIs) represent additional active options. Emerging trials suggest that combination strategies may enhance efficacy. Novel drugs, including bispecific antibodies and antibody-drug conjugates, are being evaluated in ongoing phase II trials. Conclusions:Despite the rarity of TETs, systemic treatment options have gradually expanded through the accumulation of evidence from small phase II studies. Future advances will require collaborative prospective trials to validate predictive biomarkers and support biomarker-enriched trial designs.
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.
Background and Objective:Surgical resection for thymic epithelial tumors has traditionally been performed via a median sternotomy or video-assisted thoracoscopic surgery (VATS). In recent years, however, robot-assisted thoracoscopic surgery (RATS) has been increasingly adopted as a minimally invasive alternative, with a growing number of reports. This review summarizes the progress achieved to date and outlines future perspectives in robot-assisted thymectomy. Methods:This narrative review was conducted through a structured literature search of PubMed, including articles published up to November 2025. The search strategy combined the terms ("thymectomy"[MeSH] OR thymectomy) AND ("video-assisted thoracoscopic surgery" OR VATS OR "robot-assisted thoracoscopic surgery" OR RATS OR "robotic surgery"). Eligible articles included original clinical studies, comparative analyses, large case series, and selected case reports evaluating robotic approaches to thymectomy. Both retrospective and prospective studies were considered. Non-English-language publications were excluded. Reference lists of relevant articles were also manually screened to identify additional pertinent studies. Given the narrative nature of this review, no formal meta-analysis was performed. Key Content and Findings:This review traces the evolution of robotic surgery in thymectomy, evaluates current evidence regarding indications and perioperative/oncologic outcomes, and examines the expanding array of surgical approaches enabled by contemporary robotic platforms. It also highlights emerging technologies and future directions that may shape the continued advancement of robotic thymectomy. By integrating these themes, the review synthesizes the existing evidence base and provides a framework for anticipating the future trajectory of this technique. Conclusions:Accumulating evidence on robot-assisted thymectomy suggests that further innovations and refinements in surgical techniques will continue to emerge. As the technology evolves and surgical expertise deepens, these advancements are expected not only to enhance operative precision and safety but also to broaden the indications of the robotic approach, potentially allowing its application to more extensive and complex procedures in the future.
A wide variety of benign and malignant epithelial tumors arise in the mediastinum. This review sheds light on recurrent molecular genetic alterations of mediastinal epithelial tumors to help the surgical pathologist become aware of molecularly defined entities that one may encounter in practice and understand relevant genetic underpinnings, with an emphasis on diagnostically useful immunohistochemical targets. We discuss the molecular landscape of thymoma, including World Health Organization (WHO) types as well as micronodular and metaplastic thymoma, and introduce current concepts in the molecular subtyping of thymic carcinoma. We present an overview of proposed molecular classification schemes of thymic epithelial neoplasms that focuses on commonly occurring molecular alterations described in The Cancer Genome Atlas and original data sources, and relate them to longitudinal prognostic and therapeutic implications. Poorly differentiated epithelial neoplasms of the mediastinum defined by recurrent molecular alterations can display diagnostically challenging morphologic and immunohistochemical profiles and are critically important to recognize due to their aggressive behavior. We conclude with a discussion of NUT carcinoma and switch/sucrose non-fermentable (SWI/SNF) complex deficient tumors, including thoracic SMARCA4-deficient undifferentiated tumor, reviewing salient histopathologic and immunohistochemical features that allow their recognition from histologic mimics and tumors with overlapping immunoprofiles. We lastly outline the current understanding of genetic events that give rise to these destructive tumors.
Background:Chemotherapy is the guideline-recommended first-line therapy for stage IVA thymomas (TMs) with pleural dissemination, but its efficacy has never been specifically assessed. We aim to evaluate the efficacy of chemotherapy, and to compare the results of surgery and chemotherapy without surgery in these patients. Methods:This is a real-world retrospective study. Consecutive patients with stage IVA TM with pleural dissemination who received chemotherapy or upfront surgery between 2007 and 2018 were enrolled. Propensity-score matching (PSM) was used to balance selection bias between groups. Objective response rate (ORR) in pleural lesions to chemotherapy and long-term outcomes including disease-control time (DCT), progression-free survival (PFS) and overall survival (OS) were assessed between groups. Results:Altogether 168 patients with stage IVA TM with pleural dissemination were enrolled. In 124 patients who received chemotherapy as the first-line treatment, ORR was only 6.5% with no complete remission observed, with 7.3% progression diseases. After PSM, surgery was associated with better prognosis than no-surgery [median DCT: 39.0 vs. 23.0 months, P<0.001; PFS, hazard ratio (HR): 0.457, 95% confidence interval (CI): 0.298-0.700, P<0.001; OS, HR: 0.402, 95% CI: 0.178-0.906, P=0.03]. Significantly more patients received surgery in the later [2016-2018] than in the earlier [2007-2015] time of the study period (72.9% vs. 44.4%, P<0.001), while Bayesian analysis indicated a probability of 94.23% that this shift in treatment was associated with improved PFS. Conclusions:Chemotherapy as the first-line treatment is far from satisfactory for stage IVA TMs with pleural dissemination and may not be appropriate as a routine recommendation. Surgery plays a more important role as an alternative option.
