Background and Objective:For patients undergoing evaluation for mediastinal/hilar adenopathy or masses suspicious for lymphoma, endobronchial ultrasonography (EBUS)-transbronchial needle aspiration (TBNA) offers a lower-risk alternative to surgical or percutaneous biopsy. While the utility of EBUS-TBNA is well established in lung cancer diagnosis, including biomarker analysis, its role in lymphoma is more limited. This is because for accurate subtyping of lymphoma, larger intact tissue specimens are needed and EBUS-TBNA can only provide cytology specimens. To address this limit, various needle sizes and/or sampling techniques such as cryobiopsy have been investigated to improve diagnostic sensitivity for lymphoma. In this review, we discuss the current evidence for these various techniques in the diagnosis of lymphoma. Methods:We conducted a narrative review by searching PubMed/MEDLINE, Embase and Google Scholar for articles published during 1980-2025 using search terms including "EBUS", "EBUS-TBNA", "endobronchial ultrasound", "bronchoscopy", "lymphoma diagnosis", "mediastinal lymphadenopathy" and "interventional pulmonology". Only articles written in English that focused on evaluation of diagnostic sensitivity, specimen adequacy, and interventional techniques for suspected lymphoma were included in our review. Key Content and Findings:Several studies suggested that EBUS-TBNA combined with appropriate immunohistochemical, flow cytometry, cytogenetic, and molecular studies can achieve an accurate lymphoma diagnosis. However, its sensitivity for de novo lymphoma remains modest. Various techniques such as cryobiopsy, bigger needles, and forceps via EBUS may improve tissue acquisition but whether this translates into improved diagnostic yield is uncertain. Conclusions:For patients undergoing evaluation for mediastinal/hilar adenopathy or masses suspicious for lymphoma, EBUS with TBNA, or with other techniques such as cryobiopsy, may be a reasonable first diagnostic step, as it is less invasive with a better safety profile than surgical excision of these lymph nodes. Prospective studies are needed to establish best practices for the use of EBUS to diagnose lymphoma.
The division of the mediastinum into specific compartments is essential for identifying, characterizing, and managing mediastinal abnormalities. The current international standard is the three-compartment model proposed by the International Thymic Malignancy Interest Group (ITMIG), which divides the mediastinum into prevascular (anterior), visceral (middle), and paravertebral (posterior) compartments. This model has replaced the traditional four-compartment system, which included the superior mediastinum, due to its simplicity, familiarity, and clearly defined boundaries. However, given the superior mediastinum's unique anatomy and distinct pathology, it remains clinically reasonable to consider it separately. Continued clinical use of the term "superior mediastinum" has produced numerous conflicting definitions, while the Japanese Association for Research on the Thymus (JART) has recently proposed a computed tomography (CT)-based four-compartment model. Disease distribution in the superior mediastinum is dominated by thyroid goiters and neurogenic tumors, while the incidence of thymic epithelial tumors (TETs) in this region varies among studies. Surgery in the superior mediastinum is challenging due to its narrow space and the presence of major blood vessels and nerves. Operative complications frequently include Horner's syndrome. Furthermore, studies suggest that superior mediastinal thymomas may demonstrate more aggressive behavior and lower survival rates than thymomas in other locations. Establishing a clear, standardized definition of the superior mediastinum would resolve current inconsistencies and facilitate the accumulation of detailed clinical data on superior mediastinal lesions. This article focuses on the superior mediastinum and discusses its classification, clinical features, surgical approaches, treatment outcomes, and future perspectives.
Background and Objective:Thymic epithelial tumors, comprising thymoma and thymic carcinoma, are rare and heterogeneous neoplasms for which high-level evidence is limited. Radiotherapy is integral across the disease continuum-from postoperative settings to definitive therapy for unresectable tumors and oligometastatic/recurrent disease. This narrative review synthesizes contemporary evidence and guidance on indications, technique, dose prescription, outcomes, and toxicity, with focused discussion of hypofractionation and stereotactic body radiotherapy (SBRT). Methods:A comprehensive PubMed search (1 November 2025) combined MeSH and free-text terms for thymic tumors and radiotherapy without date restriction. English-language human studies of any design were eligible. Titles/abstracts and full texts were screened independently by the author; reference lists of key studies and recent reviews were hand-searched to ensure completeness. Key Content and Findings:For completely resected early-stage thymoma, surgery alone often suffices, whereas postoperative radiotherapy (PORT) is generally favored for positive margins (R1/R2), advanced stages (III-IV) and unfavorable pathological finding. Meta-analyses increasingly suggest overall survival benefit from PORT in stage III-IV thymoma and in thymic carcinoma, with more variable findings for stage II disease. In thymic carcinoma, multi-institutional series demonstrate a clearer association between PORT and improved survival. For unresectable disease, concurrent chemoradiation using modern conformal techniques achieves high response rates and meaningful survival. Dose recommendations across international guidelines converge on 45-50 Gy (R0), 50-54 Gy (R1), and 60-70 Gy (R2), with elective nodal irradiation generally discouraged. Conclusions:Current data support PORT for high-risk features and thymic carcinoma, definitive chemoradiation for unresectable disease, and carefully selected use of hypofractionation/SBRT for limited recurrences. Priorities include randomized evaluation of PORT in borderline settings, harmonized reporting of toxicity and quality of life, and prospective study of modality selection to optimize cure while minimizing late effects.
Background and Objective:Numerous reviews have been published in the field of tracheal replacement. However, most did not differentiate between patch tracheal reconstruction (PTR), non-circumferential tracheal replacement (NCTR), and full-circumferential tracheal replacement (FCTR). This distinction is essential in terms of postoperative mortality. So far, the most widely used tracheal substitutes are the cartilage-reinforced forearm free flap (FFF) and the cryopreserved aortic allograft (CAA), respectively. The aim of our study is to identify and report the results of both preclinical and human use of the aortic grafts in tracheal replacement with a focus on the benefit/risk assessment in clinical practice. Methods:We performed an in-depth literature search with no language restriction (PubMed/MEDLINE) in order to identify articles related to the topic up to September 2025. Articles focused on experimental and human use of aortic grafts for tracheal replacement, including the authors' experience, were selected for the present review and reported in a narrative fashion. Key Content and Findings:In the setting of preclinical studies, we identified 22 articles and one relevant abstract from 1958 to 2025. In the field of human use, we retrieved 20 articles from 1999 to 2025. From a structural viewpoint, our studies of preclinical and human use of aortic grafts implanted into the trachea show that they transform into flaccid tube-shaped scar tissue that contracts over time around the luminal support up to an average of 60%. Occasionally, the graft is subject to endochondral ossification. In the clinical setting, we highlight the usefulness of aortic allograft in the case of tracheal anastomosis dehiscence. In contrast, due to the significant difference between partial and FCTR with a postoperative mortality rate of 0 vs. 18.9%, we have some reservations about the implementation of the latter procedure, mainly in the field of low-grade radiosensitive malignancies such as adenoid cystic carcinomas (ACCs). Conclusions:Physicians must be aware that the ideal tracheal substitute has not yet been discovered before considering a potential FCTR, mainly in oncology.
Thymic carcinoma (TC) is a rare, aggressive subset of thymic epithelial tumors (TETs) with a poor prognosis and limited treatment options. Representing approximately 15-20% of TETs, TC is distinct from thymoma in its histopathologic features, lack of paraneoplastic autoimmune syndromes, and more complex genomic landscape. Surgical resection remains the primary modality for early-stage disease, while platinum-based chemotherapy forms the cornerstone of treatment for advanced or unresectable TC. Advancements in targeted therapies have expanded therapeutic options, resulting in improved clinical efficacy, especially in tumors with high angiogenic activity or specific mutations (e.g., Kit). Immune checkpoint blockade (ICB) has shown activity in TC, with response rates around 20%, and is being explored in combination with chemotherapy, anti-angiogenic agents, and CTLA-4 blockade. Combinatorial strategies have demonstrated enhanced response rates but require vigilant management of immune-related adverse events. Novel therapeutic approaches are emerging, including PRMT5 inhibitors in MTAP-deficient tumors, TROP-2-directed antibody-drug conjugates (e.g., sacituzumab govitecan), and chimeric antigen receptor (CAR) T-cell therapies targeting mesothelin. Bispecific agents such as bintrafusp alfa and ivonescimab, which co-target various pathways, offer innovative strategies. Despite these advances, TC remains a challenging malignancy with no standardized treatment algorithm. Collaborative efforts across institutions will be essential to accelerate progress and improve outcomes in this rare disease.
Background:Thymoma is a rare mediastinal neoplasm derived from thymic epithelial cells and is frequently associated with paraneoplastic autoimmune syndromes. Beyond these classical manifestations, thymomas can induce chronic systemic inflammation through impaired central tolerance, cytokine release, and immune dysregulation. This inflammatory microenvironment has been increasingly recognized as a potential contributor to secondary malignancies, although reports remain scarce. Case Description:We report a 71-year-old male with a significant smoking history who was diagnosed with non-small cell lung carcinoma (NSCLC), adenocarcinoma subtype, harboring an ERBB2 mutation and programmed death ligand 1 (PD-L1) expression of 0%. Staging studies revealed a small anterior mediastinal mass initially interpreted as a metastatic lesion from NSCLC. However, biopsy performed for staging purposes demonstrated type A thymoma. Histology demonstrated spindle-shaped epithelial cells (AE1/AE3+, p40+) within a background rich in CD3+ T-cells, terminal deoxynucleotidyl transferase (TdT)+ and CD1a+ immature thymocytes, and CD20+ B-cell aggregates. The patient developed spontaneous acute pericarditis with elevated inflammatory markers, while infectious and autoimmune causes were excluded. Flow cytometry and T-cell receptor (TCR) gene rearrangement studies were negative, indicating unexplained inflammation in the context of thymoma. We hypothesize that thymoma-associated immune dysregulation contributed to lung carcinogenesis through cytokine-driven inflammation and impaired autoimmune regulator (AIRE)-mediated central tolerance, leading to reduced tumor immunosurveillance. Prior reports describing elevated pro-inflammatory cytokines in thymic lesions and lung cancer support the concept of a paracrine inflammatory niche. Conclusions:This case highlights a potential mechanistic link between thymic-associated immune dysregulation, chronic inflammation, and secondary malignancies. Recognition of this relationship is important for clinical management, long-term surveillance, and therapeutic decision-making in patients with thymoma. Future studies are warranted to further define the oncogenic consequences of thymic dysfunction and its implications for cancer development.
Background and Objective:Thymic epithelial tumors (TETs) are rare neoplasms originating from thymic epithelial cells (TECs). They exhibit histological, molecular, and immunological diversity shaped by aberrant interactions between neoplastic TECs and immune cells. World Health Organization (WHO) histological classification effectively captures their clinicopathological features, whereas inter- and intra-subtype heterogeneity persists. In this point, single-cell transcriptomics is well-suited to resolve cellular heterogeneity in TETs. This narrative review synthesizes findings from recent single-cell studies that illuminate epithelial subpopulations, intra-tumoral immune landscape, and disease-specific aberrations such as myasthenia gravis (MG)-associated phenotypes in TETs. Methods:We conducted a focused narrative review on three key studies that applied single-cell RNA sequencing (scRNA-seq) to primary human TETs. Key Content and Findings:Single-cell analyses identified a distinct TEC subpopulation ectopically expressing neuromuscular antigens, termed neuromuscular medullary TECs (nmTECs), in MG-associated thymomas, implicating aberrant antigen presentation and immune cell recruitment in autoantibody production. Other studies delineated tumor epithelial programs that shape the immune microenvironment, linking TEC transcriptional identities to the persistence of immature T-cells or dominance of mature T-cells. Bioinformatic approaches of ligand-receptor networks supported pathogenic crosstalk between neoplastic TECs and immune cells. Notably, independent groups converged on functionally oriented classifications that align across studies, stratifying TETs by epithelial lineage programs and immune composition with correspondence to biological behavior and prognosis. Conclusions:Single-cell approaches have revealed TET heterogeneity encompassing abnormal epithelial cell states, immune development trajectories, and autoimmune-associated molecular programs with high resolution. The ongoing accumulation of single-cell transcriptomics datasets would refine a cell-centric framework for TET classification and pathogenesis, while also deepening insight into normal TEC biology.
Background and Objective:Thymic epithelial tumors (TETs) require precise preoperative assessment to achieve complete resection and optimal long-term outcomes. With the expanding use of minimally invasive surgery, imaging has become central not only for diagnosis but also for surgical decision-making. This narrative review aims to summarize current evidence on how metabolic, morphologic, and computed tomography (CT)-based imaging parameters can be integrated to guide surgical strategies for TETs. Methods:A comprehensive literature search was conducted using PubMed and Web of Science for studies published between January 2001 and August 2025. Original clinical studies, multicenter analyses, systematic reviews, and major guidelines published in English were reviewed, with an emphasis on imaging-based surgical decision-making. Key Content and Findings:Tumor size (TS) consistently predicts invasiveness and surgical complexity, with thresholds of approximately 5-6 cm influencing the choice between minimally invasive and open approaches. Positron emission tomography-derived parameters, particularly maximum standardized uptake value (SUVmax) and the SUVmax-to-TS ratio, correlate with histological aggressiveness and prognosis. CT features, including capsular disruption, calcification, and vascular abutment, further refine risk stratification. Emerging modalities such as volumetry, novel positron-emission tomography (PET) tracers, and artificial intelligence (AI)-based radiomics show promise in enhancing individualized surgical planning. Conclusions:Integrating PET metrics, TS, and CT features provides a comprehensive framework for tailoring surgical strategies in TETs. Future incorporation of volumetric and AI-driven imaging analyses into clinical guidelines may further optimize surgical outcomes and personalized care.
Background and Objective:Bronchoscopic diagnosis of malignant lymphoma is challenging, because bronchoscopic tissues are often relatively small. Recently, a 19-gauge needle (19G) was developed to obtain larger tissue cores; however, its effectiveness in improving the diagnostic yields remains controversial. Therefore, in this narrative review, we aimed to summarize recent findings and evaluate the usefulness of different needle sizes for diagnosing malignant lymphoma. Methods:A comprehensive and systematic online literature search of PubMed was conducted using the keywords ("malignant lymphoma" OR "lymphoma") and ("endobronchial ultrasound" OR "endobronchial ultrasound guided" OR "endobronchial ultrasound guided transbronchial needle aspiration" OR "EBUS" OR "EBUS-TBNA" OR "needle size"). Key Content and Findings:Three prospective and 13 retrospective studies were included. Approximately 50% of the procedures were performed under moderate sedation, and the remainder under general anesthesia. Regarding subtype determination, the diagnostic yield of endobronchial ultrasound-guided transbronchial needle aspiration (EBUS-TBNA) ranged from 50% to 65% with a 19G, and from 19% to 88% with a 21G and 22G. Supportive methods such as rapid on-site cytological evaluation (ROSE) and flow cytometry were performed variably, depending on the bronchoscopist's preference or institutional resources. Conclusions:When ROSE and flow cytometry are available, there seems to be no significant difference in diagnostic yield for lymphoma subtype determination between 19G and 21/22G during EBUS-TBNA.
Background and Objective:Hepatic metastases secondary to thymic epithelial tumors (TETs), encompassing thymomas and thymic carcinomas (TCs), are a rare but clinically meaningful phenomenon and are associated with worse overall survival (OS). Management of liver metastases is largely limited to systemic therapy and chemotherapeutic regimens; although, there are smaller-scale studies in the literature reporting use of more novel treatment regimens and liver-directed therapies. The purpose of this article is to explore management options for TET hepatic metastases, including novel therapeutic options. Methods:A review of the available electronic literature was performed through October 10, 2025, using PubMed and Google Scholar. A single author identified the studies, and both authors reviewed the selection to determine which studies to include. Key Content and Findings:The literature involving systemic therapy options was much more developed, as this is the first-line treatment option for TET hepatic metastases. Smaller series supporting the use of surgical resection, radiation therapy, and interventional radiology therapies suggest benefit in liver-directed resection and/or therapies. Conclusions:Data evaluating therapeutic options for managing liver metastases secondary to TETs is limited. Given the prognostic implications of these metastatic lesions, a broader understanding of available therapies is essential for determining optimal management.
Background and Objective:Thymic epithelial tumors (TETs), encompassing thymomas (TMs) and thymic carcinomas (TCs), are rare and heterogeneous mediastinal malignancies with variable clinical behaviors and prognoses. Current prognostic assessment primarily relies on histological classification (WHO) and anatomical staging systems (Masaoka-Koga, TNM). However, the rarity and complex biology of TETs necessitate the identification of novel prognostic and predictive biomarkers to improve risk stratification and guide personalized treatment strategies. This narrative review aims to summarize and discuss emerging prognostic and predictive biomarkers in TETs beyond traditional staging systems. Methods:For this narrative review, we searched EMBASE and MEDLINE up to 4 September 2025. The terms used in the search included TM, TC, TETs, prognosis and predictive biomarkers. Key Content and Findings:Traditional staging systems (Masaoka-Koga, TNM) and histological classification retain strong prognostic value. Clinical factors (including age, resection status, and lymph node involvement) further refine risk stratification. Molecular markers such as programmed death-ligand 1 (PD-L1) expression, tumor mutational burden (TMB), DNA methylation profiles, Hippo pathway components, and Ki-67 show promise as prognostic and/or predictive biomarkers, although prospective validation remains limited. Predictive biomarkers for immunotherapy and targeted agents are under active investigation, with preliminary evidence supporting the role of TMB, PD-L1 expression, and c-kit mutations. Conclusions:Prognosis in TETs relies primarily on histology and staging, whereas molecular and immunological biomarkers represent emerging tools for risk stratification and treatment selection. Multiparametric models integrating clinical, pathological, and molecular data may pave the way for precision oncology in TETs.
Background and Objective:Thymic epithelial tumors (TETs) are a rare group of neoplasms situated in the prevascular mediastinum. Due to their rarity and histological diversity, understanding their development is quite challenging, and their molecular profile is not well defined. Although advances in molecular profiling have improved our understanding of many solid cancers, TET remains poorly characterized. The human epidermal growth factor receptor 2 (HER2/ERBB2) has gained interest as a molecular target due to its role in oncogenesis and its therapeutic significance in various malignancies; however, its potential role in treating TET remains unclear. This review examines the expression patterns, gene amplification status, and clinical implications of HER2 in thymomas and thymic carcinomas, especially in the era of antibody-drug conjugates which target tumors with low HER2 expression. Methods:PubMed, Embase and Web of Science research was conducted using the terms [HER2] OR [ERBB2] OR [ErbB2] OR [HER2/neu] OR [c-erB2] AND [thymic epithelial tumors], [HER2] OR [ERBB2] OR [ErbB2] OR [HER2/neu] OR [c-erB2] AND [thymoma], and [HER2] OR [ERBB2] OR [ErbB2] OR [HER2/neu] OR [c-erB2] AND [thymic carcinoma]. There were no restrictions on publication date. After analysis, nine articles met the research criteria. Key Content and Findings:In one study, thymic carcinomas expressed HER2 frequently, with focal to strong membranous positivity in 8 of the 17 cases. In another, HER2 was expressed in 58.3% of cases with other members of the receptor family and signaling pathway ligands also demonstrating frequent expression. The immunohistochemical analysis revealed that the receptors epidermal growth factor receptor (EGFR) (33.3%), phosphorylated epidermal growth factor receptor (pEGFR) (33.3%), and human epidermal growth factor receptor 3 (HER3) (45.8%), along with the ligands transforming growth factor-α (TGF-α) (54.2%), amphiregulin (25.0%), and epiregulin (91.7%), were expressed in the tumor cells. Only one case showed HER2 gene amplification. In another study, HER2 expression was positive in only one case of thymic carcinoma (1/22), whereas there was no expression observed in any of the thymoma cases (0/22). In the other series, there were scarce or no cases with HER2 expression or gene amplification. Conclusions:HER2 plays a still undefined role in TET, especially in thymic carcinoma. Although gene amplification is rare, protein expression is more frequent, suggesting potential for targeted therapy. Detailed molecular profiling and innovative research methods are essential to further explore the therapeutic potential of HER2 in these rare cancers.
Background and Objective:According to the National Comprehensive Cancer Network (NCCN) and other international guidelines, thymectomy is the standard surgical procedure for early-stage thymic epithelial tumors (TETs). However, the optimal extent of resection remains controversial. Recent evidence suggests that partial thymectomy, which preserves part of the thymus, may achieve outcomes comparable to thymectomy in the short- and long-term. This review aims to compare perioperative and long-term oncologic outcomes between complete and partial thymectomy in early-stage TETs. Methods:We conducted a PubMed search for English-language studies published from January 1, 1980, to July 31, 2025 that reported perioperative and long-term outcomes of patients with early-stage TETs who underwent either thymectomy or partial thymectomy. Key Content and Findings:Initial single-institution retrospective studies with limited cases were followed by larger multicenter investigations, national database analyses, and two meta-analyses. Partial thymectomy was frequently associated with shorter operative time, less blood loss, and, in some studies, lower complication rates. Short-term survival outcomes were generally similar between the two procedures. Recurrence rates, disease-free survival, and overall survival (OS) were broadly comparable, although some studies indicated slightly better OS with thymectomy in certain subgroups. Conclusions:Based on the currently available retrospective evidence, partial thymectomy may be feasible in carefully selected patients with early-stage thymoma; however, the lack of prospective data precludes definitive conclusions regarding oncologic equivalence. Most existing evidence is based on open approaches, particularly median sternotomy. Whether these findings extend to minimally invasive techniques such as video-assisted thoracoscopic surgery or robot-assisted thoracic surgery remains uncertain. Long-term data beyond 5 years are limited, and evidence regarding thymic malignancies other than thymoma is sparse. Further contemporary studies, particularly of minimally invasive approaches, are needed to clarify the relative benefits of partial thymectomy versus thymectomy.
Mediastinal parathyroid adenomas (MPAs) represent an uncommon but clinically important cause of persistent or recurrent primary hyperparathyroidism. Though most cases of sporadic primary hyperparathyroidism are due to a parathyroid adenoma in the cervical area; ectopic glands account for a fifth of cases, with only 1-2% situated in the mediastinum. Aberrant embryological descent of the inferior parathyroid glands along the thymic descent pathway gives rise to MPAs, with variable locations within the anterior or posterior mediastinum. The anatomical variability, coupled with limited accessibility is a major contributor to diagnostic uncertainty and surgical failure, especially in reoperative cases. Accurate preoperative localization using multimodal imaging is essential for optimal management of the condition. Whilst the dual modalities of imaging, namely the ultrasound scan and technetium-99m sestamibi scintigraphy are useful in cervical lesions, the sensitivity of picking up adenomas especially the small and cystic lesions in the mediastinum is quite low. Four-dimensional computed tomography (4D-CT) provides high spatial resolution and characteristic enhancement kinetics that are particularly useful in ectopic or reoperative settings, albeit at the cost of higher radiation exposure. Positron emission tomography/computed tomography (PET-CT) using 18F-fluorocholine demonstrates high sensitivity in negative or discordant first-line imaging and is increasingly adopted where available. Where non-invasive modalities fail, invasive techniques like selective venous sampling may localize mediastinal lesions but requires expertise and careful interpretation of variant venous drainage. Intervention with surgical excision via the cervical approach is possible when the MPAs are located above the innominate vein. Historically the deeper locations required access with sternotomy or thoracotomy, but with the advent of minimally invasive thoracic approaches, higher cure rates with minimal morbidity are possible, coupled with adjuncts such as intraoperative parathyroid hormone monitoring (IOPTH) and fluorescence techniques. Ongoing challenges include discordant imaging, multiglandular disease, resource limitations, and reoperative complexity, underscoring the need for stepwise imaging escalation and multidisciplinary approach to optimize outcomes.
Background and Objective:Atypical type A thymoma is a rare subtype of thymoma recently recognized for its more progressive clinical course and biological behavior, in contrast to the conventionally indolent course and favorable prognosis of conventional type A thymoma. Histologically, atypical type A thymoma retains the spindle or oval epithelial cell morphology of the conventional type, but is characterized by atypical features such as increased mitotic counts, hypercellularity, and focal necrosis, which are generally absent in the conventional type. This review integrates the latest knowledge on clinicopathological features, diagnostic criteria, and treatment. Methods:A literature search was performed using PubMed for articles in English published between January 1990 and July 2025. We included case reports, case series, and original studies with comprehensive clinicopathological and survival data for atypical type A thymomas. Key Content and Findings:We identified 67 cases of atypical type A thymoma in 16 case reports/series and two original reports that specifically mentioned detailed information for each case. Recurrence was observed in 27.3% of patients, predominantly manifesting as distant metastasis to sites such as the lungs, the rib/chest wall, and brain, a rate notably higher than that observed in conventional type A and AB thymomas. In contrast, the 5-year overall survival (OS) rate was 91.9%, indicating a prognosis closer to that of conventional type A and AB thymomas than to type B2-B3 thymomas. Its proliferative capacity, indicated by a median Ki-67 index of 10-14.5%, is closer to that of type B3 thymoma or thymic carcinoma (TC). Conclusions:Since current studies on metastatic type A thymomas often mix conventional and atypical cases, further case accumulation and the establishment of strict, standardized pathological criteria are crucial for a clearer understanding of this rare but potentially progressive tumor and for refining long-term follow-up and treatment strategies. Moreover, the relationship between metastatic and atypical type A thymomas needs to be clarified.
Background:Although some evidence shows an increase in its incidence, epithelial thymic tumors that comprise thymomas, thymic neuroendocrine tumors and thymic carcinomas, are rare (ranging from 2.5-2.8 per million). Thymomas, as presented in this paper, although slow growing lesions are malignant tumors, as the potential to invade surrounding structures and metastasize has been proven. In this case report, we present another feature of these neoplasms, recurrence. Although local and regional are far more frequent, distant can also occur. We present a case of late extra-thoracic recurrence associated with seeding from a preoperative biopsy, a very rare, but real scenario, that needs to be taken into account when deciding the need for invasive staging. Case Description:We present a case of a 75-year-old woman who was sent for evaluation of painful palpable extra-thoracic nodules, 8 years after a type A thymoma excision. After image reconstruction, surgical exploration and detailed clinical history and record examination, it was considered as recurrence associated with seeding from preoperative biopsy. She had been followed with regular computed thoracic tomography imaging, with no signs of recurrence during the 5-year period, at the moment of oncological follow-up discharge. Conclusions:This case is a statement for the changes that have been made in diagnosis, treatment and follow-up of epithelial thymic tumors in the last years. Nowadays, the biopsy is not proposed when a resectable epithelial tumor of the mediastinum is the probable diagnosis, as the case presented. We believe that a longer follow-up for these patients is needed as they can recur later than the initially considered 5-year period, and the absence of surgical excision of the biopsy tract or in the presence of a complex biopsy (involving, for example, pleural puncture), should possibly be considered a pseudo-R0.
Background:Outpatient follow-up using computed tomography (CT) after thymoma resection is recommended to evaluate tumor recurrence and detect second malignancies. However, the optimal interval for CT surveillance remains unclear. We aimed to investigate the association between CT surveillance intervals and postoperative survival after thymoma resection. Methods:This retrospective study analyzed 143 patients who underwent periodic CT scans after complete thymoma resection at a single institution between 2006 and 2022. Patients were stratified into three groups based on CT interval: half-, 1-, and 2-year groups. CT intervals were stratified according to the attending physician's clinical judgment. Overall survival (OS), recurrence-free survival (RFS), tumor recurrence rates, and post-recurrence treatments were compared among the three interval groups. A multivariate analysis was performed to evaluate the independent prognostic impact of CT intervals. Results:Among the 143 patients included, 82 were assigned to the half-year group, 33 to the 1-year group, and 28 to the 2-year group. The 7-year OS rates were 96.1% in the half-year group, 100% in the 1-year group, and 100% in the 2-year group (P=0.34). The 7-year RFS rates were 85.2%, 92.3%, and 88.1% in the half-, 1-, and 2-year groups, respectively (P=0.37). No significant differences were found in overall and RFS among the three groups. Tumor recurrence rates were 11.0% in the half-year group, 3.0% in the 1-year group, and 7.1% in the 2-year group (P=0.38). No significant differences were noted in recurrence detection modalities and treatment approaches among the three groups (P>0.99, 0.99). In the multivariate analysis, CT follow-up interval was not significantly associated with OS. Conclusions:Postoperative CT follow-up at intervals longer than 1 year may be acceptable and feasible for patients after thymoma resection.
Thymic epithelial tumors (TETs) are rare cancers that arise from cortical or medullary thymic epithelial cells and are associated with unique clinical, genomic and immunological features that include a poorly understood predisposition towards autoimmunity. The risk for autoimmune disease can be traced back to the physiological role of the thymus in T cell development. Recent studies have revealed key differences in the immune cell composition and tumor immune microenvironment of thymomas and thymic carcinomas (TCs). Thymomas contain a greater proportion of immature double positive T cells whereas TCs have larger numbers of differentiated single positive T cells. The proportion of B cells, dendritic cells, macrophages and regulatory T cells vary depending on TET histology. Thymomas exhibit higher immune cell infiltration whereas TCs are characterized by a pronounced stromal signature. Histology-based differences in T cell receptor diversity and in the cytokine profile of the tumor microenvironment have also been described. Programmed cell death-ligand 1 expression is variable in TETs and confounded by physiological expression in the normal thymus. Knowledge of the immune cell composition and the tumor immune microenvironment of TETs is essential to understand the risks of paraneoplastic autoimmunity and treatment-related toxicity in the era of immunotherapy. This review synthesizes recent advances in thymic biology and tumor immunology to frame the immune landscape of TETs.
Background:Thymomas are thymic epithelial tumors (TETs) and the most common tumors of the anterior mediastinum. In the natural course of this malignancy, there is no tendency towards extrathoracic metastasis. This report describes an extremely rare case of splenic metastatic disease from a thymoma. Additionally, the patient developed Good's syndrome, a thymoma-associated immunological disorder which makes the patient vulnerable for opportunistic infections. This syndrome is generally treated with intravenous immunoglobulins. Case Description:The patient is an asymptomatic 73-year-old man of Caucasian origin without significant past medical history. He was diagnosed with an anterior mediastinal mass on computed tomography (CT) of the chest in the work-up of his newly diagnosed prostate carcinoma, which turned out to be a thymoma after surgical resection. During follow-up the patient developed a splenic lesion which was difficult to characterize. After laparoscopic splenectomy the diagnosis of metastatic thymoma was confirmed. Additionally, the patient developed Good's syndrome. Unfortunately, the patient died of associated infectious complications after thorough intensive care treatment. Conclusions:Metastatic splenic disease from a thymoma, further complicated by development of Good's syndrome is extremely rare and hitherto never described before. To anticipate possible infectious complications in patients with newly diagnosed thymoma, we advise to routinely check immunoglobulin levels in these patients.
Background and Objective:Thymic carcinoma is a type of thymic epithelial tumor that is rare and aggressive. Compared to patients with thymoma, patients with thymic carcinoma experience worse overall survival, progression-free survival and earlier relapses. Patients with stage IV thymic carcinoma most commonly have disease involving the pleural or pericardium, but distant spread outside the thorax is also possible. The purpose of this review is to describe radiation therapy strategies and options for patients with stage IV thymic carcinoma. Methods:Due to the rare nature of thymic tumors, the literature on stage IV thymic carcinoma is limited. As a result, most of the data on stage IV thymic carcinoma is based on retrospective reviews and heterogenous populations. Key Content and Findings:The management of stage IV thymic carcinoma is multidisciplinary and depends on many factors including the patient's performance status, extent of disease, biology of the disease and intent of treatment. Systemic therapy, surgical resection, observation, and radiation therapy are often considered. Radiation can be used for palliation of symptoms, prevention of impending symptoms, and potentially for prolongation of disease control particularly in patients with oligometastatic or oligoprogressive disease. The type of radiation used in patients with stage IV thymic carcinoma depends on the intent of treatment, location of the disease, size of the target, and proximity to organs that are sensitive to radiation. Various techniques such as 3D conformal radiation therapy (3DCRT), stereotactic body radiation therapy (SBRT), intensity modulated radiation therapy (IMRT), proton beam therapy, and hemi-thoracic intensity-modulated pleural radiation therapy (IMPRINT) may be considered depending on these factors. Conclusions:In patients with stage IV thymic carcinoma, radiation therapy is an effective modality for palliation of symptoms, prevention of impending symptoms, and potentially for prolongation of disease control. Due to the rare nature of the disease, additional data and research are required to better understand the impact and appropriateness of various treatment approaches in patients with thymic carcinoma. Because the management of stage IV thymic carcinoma can involve multiple different modalities depending on the patient's individual case, a multidisciplinary approach is critical for optimal patient care.
