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Introduction: Thymic epithelial tumors represent the most common cause of anterior mediastinal masses in adults. Among these tumors, thymomas constitute the majority, whereas thymic carcinomas are less prevalent and associated with a poorer prognosis. This study seeks to investigate the clinical and prognostic factors of these rare entities, thymoma, and thymic carcinomas. Materials and methods: We conducted a retrospective analysis on a cohort of 60 patients diagnosed with thymoma and thymic carcinoma, who were under follow-up at our clinic between January 1998 and February 2023. The clinical characteristics and prognostic factors of these patients were analyzed separately. Results: Thymomas accounted for 75% of the cases. The median age at diagnosis was 46 years in both patient groups. Masaoka stage 4 was observed in 28.9% of thymoma patients and 66.7% of thymic carcinoma patients. The median overall survival (mOS) for thymoma patients was 261.4 months, while it was 9.23 months for patients with thymic carcinoma. Curative surgery emerged as a prognostic factor significantly influencing overall survival in both thymoma and thymic carcinoma patients. Conclusion: Our study highlights the significance of Eastern Cooperative Oncology Group (ECOG) performance status and curative surgery as prognostic factors affecting overall survival in thymoma patients. In thymic carcinoma, only curative surgery was found as a prognostic factor. These findings may enhance patient care and guide personalized treatment strategies. Further investigations and prospective studies are warranted to corroborate and expand upon these results. [ABSTRACT FROM AUTHOR]

T‐lymphoblastic lymphoma (T‐LBL) and thymoma are two rare primary tumors of the thymus deriving either from T‐cell precursors or from thymic epithelial cells, respectively. Some thymoma subtypes (AB, B1, and B2) display numerous reactive terminal deoxynucleotidyl transferase‐positive (TdT+) T‐cell precursors masking epithelial tumor cells. Therefore, the differential diagnosis between T‐LBL and TdT+ T‐lymphocyte‐rich thymoma could be challenging, especially in the case of needle biopsy. To distinguish between T‐LBL and thymoma‐associated lymphoid proliferations, we analyzed the global DNA methylation using two different technologies, namely MeDIP array and EPIC array, in independent samples series [17 T‐LBLs compared with one TdT+ lymphocyte‐rich thymoma (B1 subtype) and three normal thymi, and seven lymphocyte‐rich thymomas compared with 24 T‐LBLs, respectively]. In unsupervised principal component analysis (PCA), T‐LBL and thymoma samples clustered separately. We identified differentially methylated regions (DMRs) using MeDIP‐array and EPIC‐array datasets and nine overlapping genes between the two datasets considering the top 100 DMRs including ZIC1, TSHZ2, CDC42BPB, RBM24, C10orf53, and MACROD2. In order to explore the DNA methylation profiles in larger series, we defined a classifier based on these six differentially methylated gene promoters, developed an MS‐MLPA assay, and demonstrated a significant differential methylation between thymomas (hypomethylated; n = 48) and T‐LBLs (hypermethylated; n = 54) (methylation ratio median 0.03 versus 0.66, respectively; p < 0.0001), with MACROD2 methylation status the most discriminating. Using a machine learning strategy, we built a prediction model trained with the EPIC‐array dataset and defined a cumulative score taking into account the weight of each feature. A score above or equal to 0.4 was predictive of T‐LBL and conversely. Applied to the MS‐MLPA dataset, this prediction model accurately predicted diagnoses of T‐LBL and thymoma. © 2024 The Author(s). The Journal of Pathology published by John Wiley & Sons Ltd on behalf of The Pathological Society of Great Britain and Ireland. [ABSTRACT FROM AUTHOR]

ABSTRACT Thymoma‐associated multiorgan autoimmunity (TAMA) is a rare paraneoplastic disorder that presents similarly to graft versus host disease (GVHD). We report a unique case of TAMA presenting as a GVHD‐like erythroderma in an elderly male with a history of benign thymoma. Cutaneous histopathological findings demonstrated vacuolar interface dermatitis with numerous dyskeratotic keratinocytes, exocytosis of lymphocytes, and a mildly acanthotic epidermis, which can be seen in several different disease processes. Thus, careful consideration of clinical presentation, historical information, laboratory testing, and histopathologic findings led to the correct diagnosis and treatment. [ABSTRACT FROM AUTHOR]

Background: Thymoma is a primary tumor of the thymus, commonly located in the anterior mediastinum. Most thymomas are benign or low‐grade malignant, but they can invade surrounding organs or metastasize. The primary treatment for thymoma is surgical resection. Traditional methods involve open thoracotomy, but it is traumatic, with slow recovery and many complications. In recent years, with the development of thoracoscopic techniques, thoracoscopic total thymectomy has gradually become the preferred method for small size thymomas due to its minimally invasive, safe, and effective. Methods: This paper introduces a thoracoscopic extend thymectomy technique, the subxiphoid video–assisted thoracoscopic extend thymectomy with sternal suspension. This method involves placing hooks at the upper and lower ends of the sternum to suspend the sternum upward, increasing the thoracic cavity space and facilitating thoracoscopic operations. This research reviews the clinical data of 59 patients with early‐stage thymomas treated with this technique at our center since 2020 and analyzes the perioperative therapeutic efficacy and safety. It also compares the outcomes with those of 17 patients who underwent thoracoscopic approaches. Results: The results show that subxiphoid video–assisted thoracoscopic total thymectomy with sternal suspension is an innovative and effective surgical method, achieving the same tumor eradication as other thoracic surgeries. The flexible switching of observation ports provides a more comprehensive surgical field, reduces surgical trauma and complications, and improves the surgical outcomes and quality of life for patients. [ABSTRACT FROM AUTHOR]

The therapeutic approach to thymic cysts remains a subject of controversy. Predicted biomarkers for identifying thymic cysts and thymoma (THYM) are crucial. In this research, patients diagnosed with thymic cysts (MTC, n = 6) and thymoma (B1, n = 6; B3, n = 6) were enrolled. Proteins of superior quality were subjected to TMT labeling and UPLC-MS, and differentially expressed proteins (DEPs) were identified. Gene Ontology (GO), Kyoto Encyclopedia of Genes and Genomes (KEGG), and protein-protein interactive network analyses were applied to the DEPs. Some key differentially expressed genes(DEGs) were corroborated through GEPIA 32. The pan-cancer expression levels of key DEGs remarkably linked with prognosis were determined utilizing The University of ALabama at Birmingham CANcer data analysis Portal (UALCAN). Eventually, 49 DEPs were identified in the B1 vs. MTC comparison (17 upregulated and 32 downregulated), 27 in the B3 vs. MTC comparison (8 upregulated and 19 downregulated), and 38 in the B3 vs. B1 comparison (9 upregulated and 29 downregulated). IL13RA1 (down), galectin-3 binding protein (LGALS3BP)(up), PRCSH (down), C3 (down), MXRA5 (down), TNN (down), CFHR1 (down), SUN3 (down) were jointly altered in both B1 vs. NZ and B3 vs. NZ. GEPIA validated that LGALS3BP was significantly upregulated in thymoma patients. In conclusion, LGALS3BP might be an essential biomarker to identify thymoma from the thymic cyst. [ABSTRACT FROM AUTHOR]

Objectives: Good's syndrome (GS) is a rare secondary immunodeficiency which is characterized by hypogammaglobulinemia and thymoma. This study aims to investigate the expression and distribution of B cells in thymoma tissue, given that B cells had been found to be reduced or absent in peripheral blood or bone marrow. Methods: This study retrospectively analyzed thymoma tissues from 5 GS patients at the First Affiliated Hospital of Wenzhou Medical University and Zhejiang Provincial People's Hospital. Tissues from 20 patients with simple thymoma were used as controls. Immunohistochemistry analyses were performed to detect the markers CD19, CD20, PAX5 and CD138 to evaluate the expression of B cells or plasma cells. Results: Compared to the control group, 4 GS patients exhibited a complete absence of B cells in their thymoma tissue, while 1 GS patient exhibited a notable reduction in B cells. The expression levels of CD19, CD20 and PAX5 in the thymoma tissues of GS patients were dramatically lower than those in the control group (0 vs. 85%, 20 vs. 85%, 20 vs. 85%, respectively; P < 0.05). However, no significant difference was observed in the expression frequency of CD138 between the thymoma tissues of the two groups (0 vs. 30%, P > 0.05). Conclusion: This is the first report of B cell deficiency in 5 thymoma tissues of GS patients. [ABSTRACT FROM AUTHOR]

A 41 year old female with stage 5 chronic kidney disease undergoing hemodialysis was admitted to the hospital. Chest CT scan revealed a large mass lesion of approximately 6.0 × 3.5x4.9 cm in size in the anterior superior mediastinum and a ground glass nodule in the upper lobe of the right lung, which increased in size from 9 × 7 mm 1 year and 9 months ago to 11mmx9mm before surgery. We designed a localization method to accurately locate the pulmonary nodule and successfully performed thoracoscopic minimally invasive resection of both thymoma and lung cancer through a subxiphoid approach with the same incision for this patient. With the support of perioperative hemodialysis, the patient's outcome is good. The pathological diagnosis of the anterior mediastinal mass is thymoma (b1 type), and the pathological diagnosis of the right upper lobe nodule is invasive lung adenocarcinoma (acinar type). This report describes the diagnosis and treatment process of the case. [ABSTRACT FROM AUTHOR]

AbstractBackground and purposeMethodsResultsConclusionPublic medical insurance data are increasingly used to study myasthenia gravis (MG); however, a validated case definition is lacking. We assessed the clinical characteristics of patients identified according to previously used case definitions.Patients with diagnosis code G70.0 (myasthenia gravis) who visited Severance Hospital between January 2019 and December 2020 were retrospectively analyzed. Three case definitions used in previous epidemiologic studies were applied to the included patients, and their performance was evaluated based on sensitivity, specificity, and positive predictive value (PPV).A total of 843 patients with diagnosis code G70.0 were identified, and 796 (96.7%) met the clinical diagnostic criteria for MG. All three previously-used case definitions showed a PPV of >98% in identifying clinically diagnosed MG, with sensitivities ranging from 68.7 to 71.1%. Case definition #1 was unable to identify 230 patients with MG: 42.6% were in remission, 23.5% were lost to follow-up, and 25.2% were receiving prescriptions elsewhere. A similar pattern was observed for definitions #2 and #3: Patients with thymoma-associated MG who were followed-up by thoracic surgeons were frequently missed based on the previously used case definitions. A proposed case definition that additionally includes a history of thymoma enhanced the sensitivity to 77.9%, while maintaining a PPV of 99.4%.The previously used case definitions showed a high PPV but were unable to identify patients in remission or followed-up elsewhere. These limitations can be overcome by utilizing a new case definition and expanding the study duration. [ABSTRACT FROM AUTHOR]

Background: Thymic pathologies represent the most common lesions of the anterior mediastinum. They may be classified as malignant or benign. Current diagnostic pathways recommend an initial assessment with computed tomography (CT) imaging to delineate potentially malignant thymic lesions. Despite this, high rates of non-therapeutic thymectomy continue to be observed. This carries with it significant anaesthetic, operative, and post-operative risks, in addition to healthcare costs. Consequently, there is a growing interest in magnetic resonance imaging (MRI) as a primary diagnostic modality for lesions of the anterior mediastinum. This narrative review outlines the current approaches to the evaluation of thymic lesions, with a discussion of the strengths and limitations of CT and MRI imaging modalities. It also evaluates the current discourse on the use of upfront MRI for thymic and anterior mediastinal lesion assessment. Methods: A narrative review was performed following a search on the Medline database. Articles that were evaluated had explored the role of MRI on the evaluation of thymic and anterior mediastinal lesions. Results: Current work-up for thymic and anterior mediastinal lesions are highly variable and centre around the use of CT. Upfront MRI demonstrates a similar accuracy to CT for various thymic and anterior mediastinal pathologies; however, the efforts to integrate this approach into routine practice remain in their infancy, with no standardised guidelines that exist. Conclusions: This narrative review demonstrates that there is a paucity of evidence relating to the sensitivity and specificity of MRI compared to CT for thymic lesion analysis and their subsequent relationship with non-therapeutic thymectomy. Future prospective trials to assess the role of MRI in thymic lesion determination are required to understand whether MRI can more accurately characterise these lesions to reduce non-therapeutic thymectomy. Additionally, further research efforts are required to characterise best-practice methods for integrating MRI into diagnostic pathways for these lesions in a cost-effective and resource-conscious manner. [ABSTRACT FROM AUTHOR]

Background: Surgical approach in the treatment of thymoma is varied. This retrospective study aimed to evaluate the outcomes of different surgical approaches in the treatment of thymoma. Methods: From January 2020 to December 2023, a total of 208 patients underwent thoracoscopic surgical treatment and were diagnosed with thymoma by postoperative pathological result in our institution. We collected and then analysed potential factors including general conditions, surgical approaches, preoperative clinical symptoms, surgical procedures, postoperative hospital stay, postoperative drainge, postoperative pain score, postoperative complications and postoperative follow-up. Results: Of the 208 patients, 98 patients underwent surgery via subxiphoid approach (47.1%) and 110 patients via lateral intercostal approach (52.8%). The duration of the surgical procedure (P = 0.02), intraoperative blood loss (P = 0.045), total postoperative drainage (P = 0.021), postoperative drainage per day (P = 0.004) and Postoperative pain score (Day1 P = 0.010 and Day2 P = 0.002)showed a significant difference between these two groups. 31 patients (14.9%) received postoperative radiotherapy. One patient died due to postoperative radiotherapy. No recurrence occurred in one-year follow-up. Conclusions: The present study not only showed that subxiphoid approach was a safe and feasible technique for thymoma, with a potentially faster postoperative recovery and less postoperative pain. [ABSTRACT FROM AUTHOR]

Introduction: Myasthenia gravis is an autoimmune disease that is charecterized by production of acetyl cholinesterase receptor auto antibodies. This is a retrospective study done in 45 patients who were offered trans-sternal thymectomy in such patients. Objectives: Adequate pre operative preparation with immunosuppressive agents and myasthenia gravis modifying drugs, plasmapheresis before surgery helps optimize out comes. Material and methods:Retrospectively data was collected from the years 2017-2020 in 45 patients who underwent trans-sternal thymectomy for myasthenia gravis and results analysed. Results: There has been no mortality noted in this study. 13 patients needed post operative management of myasthenic crisis. There was no reduction in the dosage of pyridostigmine. However, there has been a significant reduction in the dosage of steroids and azathriopine. Conclusion: Presence of thymoma, positive AChR Antibodies, moderate to severe airway disease are significant predictors of perioperative crisis. Prior adequate optimization before surgery is of utmost importance to prevent postoperative myasthenia crisis. Thymectomy is a very safe and effective procedure. [ABSTRACT FROM AUTHOR]

Thymoma is a relatively uncommon thoracic solid tumor, and considered to possess malignant potential. Usually, the lung, pleura, and mediastinum are the most frequently affected sites for metastasis in thymoma. However, the thymoma presenting simultaneous intrathoracic and extrathoracic metastases are exceedingly rare. Herein, we present an exceptionally uncommon case of invasive thymoma with multiple metastases. Furthermore, our case underscores the indispensable role of multimodality imaging in confirming the primary diagnosis and guiding treatment decisions. [ABSTRACT FROM AUTHOR]

Background: Thymoma is a tumor that originates in the thymus gland, a part of the human body located behind the breastbone. It is a malignant disease that is rare in children but more common in adults and usually does not spread outside the thymus. The exact cause of thymic disease is not known, but it is thought to be more common in people infected with the EBV virus at an early age. Various surgical methods are used in clinical settings to treat thymoma. Expert opinion is very important in the diagnosis of the disease. Recently, next-generation technologies have become increasingly important in disease detection. Today's early detection systems already use transformer models that are open to technological advances. Methods: What makes this study different is the use of transformer models instead of traditional deep learning models. The data used in this study were obtained from patients undergoing treatment at Fırat University, Department of Thoracic Surgery. The dataset consisted of two types of classes: thymoma disease images and non-thymoma disease images. The proposed approach consists of preprocessing, model training, feature extraction, feature set fusion between models, efficient feature selection, and classification. In the preprocessing step, unnecessary regions of the images were cropped, and the region of interest (ROI) technique was applied. Four types of transformer models (Deit3, Maxvit, Swin, and ViT) were used for model training. As a result of the training of the models, the feature sets obtained from the best three models were merged between the models (Deit3 and Swin, Deit3 and ViT, Deit3 and ViT, Swin and ViT, and Deit3 and Swin and ViT). The combined feature set of the model (Deit3 and ViT) that gave the best performance with fewer features was analyzed using the mRMR feature selection method. The SVM method was used in the classification process. Results: With the mRMR feature selection method, 100% overall accuracy was achieved with feature sets containing fewer features. The cross-validation technique was used to verify the overall accuracy of the proposed approach and 99.22% overall accuracy was achieved in the analysis with this technique. Conclusions: These findings emphasize the added value of the proposed approach in the detection of thymoma. [ABSTRACT FROM AUTHOR]

Thymus is a paired organ which develops during the sixth week of fetal life, attains maximum size by three years and involutes in adult life. Anatomically it is situated in superior mediastinum. Thymus can be present at ectopic sites like neck, skull base, middle ear, submandibular gland and tonsil. Ectopic Cervical Thymus (ECT) is very rare in adults and can present as a solid mass, cyst or thymoma. ECT occurs due to failure of descent during embryonic development. ECT can mimic any pathologic mass, metastatic deposit or lymph node enlargement. We report a case of ECT discovered incidentally in an adult during histopathological examination of thyroid carcinoma. In addition, we described the role of immunohistochemistry markers to confirm the various thymic components. [ABSTRACT FROM AUTHOR]

Purpose: Thoracoscopic procedures such as video-assisted thoracoscopic surgery (VATS) and robot-assisted thoracoscopic surgery (RATS) have gained popularity for the treatment of thymoma. Accurate preoperative assessments of tumor invasion are crucial to identifying the appropriate surgical approach. Although imaging techniques have been used to predict invasion, a quantifiable method is still needed in clinical practice. Methods: The ubjects of this retrospective study were 226 patients with thymoma who underwent surgery at our hospital. Clinicopathological data, tumor staging, and recurrence rates were analyzed. Calcification identified through computed tomography (CT) defined the "calcified group" as having a long diameter of ≥ 5 mm. Statistical analyses were performed to assess relationships and survival outcomes. Results: The calcified group had higher Masaoka and World Health Organization classification than the noncalcified group, with significantly higher organ invasion rates. The calcified group also had remarkably higher recurrence rates. Conclusion: Thymoma calcification appears to correlate with increased invasiveness and recurrence rates, suggesting its potential as a predictor of tumor stage and prognosis. Despite its retrospective nature and inherent limitations, this study highlights the potential clinical significance of calcification in the surgical planning and prognostication of patients with thymoma. [ABSTRACT FROM AUTHOR]

Background: Thymoma presents with several autoimmune manifestations and is associated with secondary autoimmune regulator (AIRE) deficiency. Pneumonitis has recently been described as an autoimmune manifestation associated with thymoma presenting with similar clinical, radiographic, histological, and autoantibody features as seen in patients with inherited AIRE deficiency who suffer from Autoimmune PolyEndocrinopathy-Candidiasis-Ectodermal Dystrophy (APECED) syndrome. Objectives: To treat two patients with biopsy-proven thymoma-associated pneumonitis with lymphocyte-directed immunomodulation. Methods: Two patients with thymoma were enrolled on IRB-approved protocols at the NIH Clinical Center. We performed history and physical examination; laboratory, radiographic, histologic and pulmonary function evaluations; and measurement of the lung-directed autoantibodies KCNRG and BPIFB1 prior to and at 1- and 6-months following initiation of lymphocyte-directed immunomodulation with azathioprine with or without rituximab. Results: Combination T- and B-lymphocyte-directed immunomodulation resulted in improvement of clinical, functional, and radiographic parameters at 6-month follow-up evaluations in both patients with sustained remission up to 12–36 months following treatment initiation. Conclusion: Lymphocyte-directed immunomodulation remitted autoimmune pneumonitis in two patients with thymoma. [ABSTRACT FROM AUTHOR]

Background: Thymoma is a key risk factor for myasthenia gravis (MG). The purpose of our study was to investigate the potential key genes responsible for MG patients with thymoma. Methods: We obtained MG and thymoma dataset from GEO database. Differentially expressed genes (DEGs) were determined and functional enrichment analyses were conducted by R packages. Weighted gene co-expression network analysis (WGCNA) was used to screen out the crucial module genes related to thymoma. Candidate genes were obtained by integrating DEGs of MG and module genes. Subsequently, we identified several candidate key genes by machine learning for diagnosing MG patients with thymoma. The nomogram and receiver operating characteristics (ROC) curves were applied to assess the diagnostic value of candidate key genes. Finally, we investigated the infiltration of immunocytes and analyzed the relationship among key genes and immune cells. Results: We obtained 337 DEGs in MG dataset and 2150 DEGs in thymoma dataset. Biological function analyses indicated that DEGs of MG and thymoma were enriched in many common pathways. Black module (containing 207 genes) analyzed by WGCNA was considered as the most correlated with thymoma. Then, 12 candidate genes were identified by intersecting with MG DEGs and thymoma module genes as potential causes of thymoma-associated MG pathogenesis. Furthermore, five candidate key genes (JAM3, MS4A4A, MS4A6A, EGR1, and FOS) were screened out through integrating least absolute shrinkage and selection operator (LASSO) regression and Random forest (RF). The nomogram and ROC curves (area under the curve from 0.833 to 0.929) suggested all five candidate key genes had high diagnostic values. Finally, we found that five key genes and immune cell infiltrations presented varying degrees of correlation. Conclusions: Our study identified five key potential pathogenic genes that predisposed thymoma to the development of MG, which provided potential diagnostic biomarkers and promising therapeutic targets for MG patients with thymoma. [ABSTRACT FROM AUTHOR]

Background: Thymomas complicated with myasthenia gravis are conventionally treated during thoracic surgery. Particularly, invasive thymomas are resected alongside the surrounding organs. Here, we present a case where surgical and perioperative management was performed under the presumption of thymoma with myasthenia gravis. However, definitive pathology revealed the co-occurrence of B3 thymoma and thymic squamous cell carcinoma. This case highlights the unique presentation and exceptional rarity of thymomas that are complicated by myasthenia gravis and thymic carcinoma. Case presentation: A 65-year-old female presented with eyelid ptosis at our hospital. Following a comprehensive examination, the patient was diagnosed with myasthenia gravis. Her computed tomography (CT) scan revealed an anterior mediastinal tumor suggestive of a thymoma, prompting a referral to the Department of Thoracic Surgery. Moreover, preoperative assessment could not definitively exclude pericardial invasion. She subsequently underwent an extended thymectomy via a longitudinal sternal incision. The tumor exhibited partial invasion of the pericardium, necessitating resection and reconstruction. Definitive pathological examination confirmed the co-occurrence of B3 thymoma and thymic squamous cell carcinoma. Positive lymph node metastasis classified the patient as stage IVa according to the Union for International Cancer Control (UICC) TNM Classification of Malignant Tumors, 8th Edition, and she was started on adjuvant radiotherapy postoperatively. Currently, the patient remains under observation, with follow-up CT scans showing no signs of recurrence. Conclusions: This report describes an extremely rare case of thymoma complicated with myasthenia gravis and thymic squamous cell carcinoma. [ABSTRACT FROM AUTHOR]

Myasthenia gravis (MG) and idiopathic inflammatory myopathy (IIM) are autoimmune diseases of the nervous system, and their main clinical manifestation is muscle weakness. The concurrent presence of both conditions in the same patient is clinically rare and easily missed. Here, we report the case of a 74-year-old woman who went to the doctor with fluctuating weakness of the limbs and muscle pain. By analyzing the patient's history and the results of repeated frequency electrical stimulation, chest computed tomography, thigh muscle magnetic resonance imaging, serum antibody detection, lymph node biopsy, etc., she was finally diagnosed with MGconcomitant IIM with squamous cell carcinoma of the thymus. Acetylcholine receptor antibody, titin antibody, ryanodine receptor antibody, anti-JO-1 antibody, and Ro-52 antibody tests were positive. MG-concomitant IIM is often associated with thymoma. The immunopathology mechanism may be different from that of pure MG or IIM, which needs further research. [ABSTRACT FROM AUTHOR]