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Cellular & Molecular Immunology May 2011The thymus is a central lymphatic organ that is responsible for many immunological functions, including the production of mature, functional T cells and the induction of... (Review)
Review
The thymus is a central lymphatic organ that is responsible for many immunological functions, including the production of mature, functional T cells and the induction of self-tolerance. Benign or malignant tumors may originate from the thymus gland, with thymoma being the most common and accounting for 50% of anterior mediastinal tumors. Malignancies linked to thymoma include the loss of self-tolerance and the presence of autoimmunity. In this review, we compiled the current scientific evidence detailing the various interactions between thymoma and autoimmune diseases, including myasthenia gravis, systemic lupus erythematosus, inappropriate antidiuretic hormone secretion, pure red cell aplasia, pernicious anemia, pemphigus and autoimmune thyroid diseases. In recent years, several mechanisms have been proposed to explain these interactions. Most are based on the assumption that the 'sick' thymus, like the 'normal' thymus, can generate mature T cells; however, the T cells generated by the sick thymus are impaired and thus may exert cellular autoreactivity. Here, we present several theories that may shed light on the loss of self-tolerance associated with this epithelial tumor of the thymus.
Topics: Animals; Autoimmune Diseases; Autoimmunity; Cell Differentiation; Humans; Self Tolerance; T-Lymphocytes; Thymoma; Thymus Gland; Thymus Neoplasms
PubMed: 21317916
DOI: 10.1038/cmi.2010.74 -
Thoracic Cancer Nov 2021A thymoma is a type of thymic tumor which is rarely malignant that is frequently reported in adult patients. A number of thymoma-related immune disorders are observed... (Review)
Review
A thymoma is a type of thymic tumor which is rarely malignant that is frequently reported in adult patients. A number of thymoma-related immune disorders are observed including autoimmune diseases, which suggests a strong connection between thymoma development and immunological mechanisms. Characterized by association with humoral and cellular immunodeficiency, thymoma patients are susceptible to opportunistic infections by environmental factors. Recent reports have suggested that viral infection may play a role in the etiological mechanisms of thymoma development associated with dysregulated immunity. In this review, we summarize the case reports and studies related to viral infection, such as CMV, EBV and HSV, that probably play a part in the pathogenesis of thymoma and related diseases. Furthermore, we demonstrate the underlying mechanisms by which viruses may induce the occurrence of thymoma with autoimmune diseases. Lastly, we discuss the potential application of antiviral therapy in the treatment of thymic diseases.
Topics: Autoimmune Diseases; Humans; Opportunistic Infections; Thymoma; Thymus Neoplasms; Virus Diseases
PubMed: 34532982
DOI: 10.1111/1759-7714.14157 -
Zhongguo Fei Ai Za Zhi = Chinese... Mar 2020Thymoma is a potential malignant disease with a recurrence rate of 10% to 30% after complete resection. There is no agreement on the treatment strategy and standard for... (Review)
Review
Thymoma is a potential malignant disease with a recurrence rate of 10% to 30% after complete resection. There is no agreement on the treatment strategy and standard for recurrent thymoma. The treatment methods include reoperation, chemotherapy, radiotherapy, targeted therapy and immunotherapy. In this article, we review the previous literature and summarize the indications, efficacy and prognosis of different treatments for recurrent thymoma, so as to provide some reference for treatment criteria for recurrent thymoma.
Topics: Humans; Prognosis; Thymoma
PubMed: 32102138
DOI: 10.3779/j.issn.1009-3419.2020.03.11 -
Asian Pacific Journal of Cancer... Mar 2020Our objectives are to investigate the clinicopathological features, treatment modalities, and prognostic and prognostic factors in order to estimate long-term outcomes...
OBJECTIVE
Our objectives are to investigate the clinicopathological features, treatment modalities, and prognostic and prognostic factors in order to estimate long-term outcomes for patients with thymoma and thymic carcinoma at our institution.
METHODS
We reviewed all patients diagnosed with thymic malignancies malignancies over a period of 38 years (from 1976 to 2014). Patients were identified using a single institution database at King Faisal Specialist Hospital and Research Center (KFSH and RC), Riyadh. Demographic data, clinical staging, histopathology classification, treatment approaches, and survival data were collected. Data Analysis was performed using both the Kaplan-Meier method and Cox proportional hazards modeling.
RESULTS
The fifty-six identified patients consists of 30 females (53.6%) and 26 males (46.4%). The median age at diagnosis was 39 years. About 37% of the patients were diagnosed with myasthenia gravis (MG). There was a significant association between the WHO histologic classification and the Masaoka stage (p= 0.018). The estimated 5-year overall survival rate was 88.6% for patients with thymic malignancies. The median survival time of thymoma and thymic carcinoma was 61 and 14 months, respectively. The univariate analysis suggested that histology (thymoma versus thymic carcinoma, p= 0.044) and Masaoka stage (II-III versus IV, p= 0.048) were independent prognostic factors affecting overall survival. Histology (p = 0.044) was found to be an independent predictor of overall survival.
CONCLUSION
The findings of this study indicates that late Masaoka-Koga staging and histology types are significantly associated with extended overall survival. Similarly, surgical resection and multimodality treatments play a significant role in thymic malignancies neoplasms therapy strategies to prolong survival rates.
Topics: Adolescent; Adult; Aged; Aged, 80 and over; Female; Humans; Male; Middle Aged; Neoplasm Staging; Prognosis; Thymoma; Thymus Neoplasms; Young Adult
PubMed: 32212790
DOI: 10.31557/APJCP.2020.21.3.653 -
International Journal of Biological... 2023As the most common malignancy from mediastinum, the metabolic reprogramming of thymoma is important in its development. Nevertheless, the connection between the...
As the most common malignancy from mediastinum, the metabolic reprogramming of thymoma is important in its development. Nevertheless, the connection between the metabolic map and thymoma development is yet to be discovered. Thymoma was categorized into three subcategories by unsupervised clustering of molecular markers for metabolic pathway presentation in the TCGA dataset. Different genes and functions enriched were demonstrated through the utilization of metabolic Gene Ontology (GO) analysis. To identify the main contributors in the development of thymic malignancy, we utilized Gene Set Enrichment Analysis (GSEA), Gene Set Variation Analysis (GSVA), and Kyoto Encyclopedia of Genes and Genomes (KEGG) pathway enrichment analysis. The prognosis of thymoma was evaluated by screening the essential pathways and genes using GSVA scores and machine learning classifiers. Furthermore, we integrated the transcriptomics findings with spectrum metabolomics investigation, detected through LC-MS/MS, in order to establish the essential controller network of metabolic reprogramming during thymoma progression. The thymoma prognosis is related to glycosphingolipid biosynthesis-lacto and neolacto series pathway, of what high B3GNT5 indicate poor survival. The investigation revealed that glycosphingolipid charts have a significant impact on metabolic dysfunction and could potentially serve as crucial targets in the clinical advancement of metabolic therapy.
Topics: Humans; Thymoma; Chromatography, Liquid; Tandem Mass Spectrometry; Thymus Neoplasms; Cluster Analysis
PubMed: 37781041
DOI: 10.7150/ijbs.83468 -
European Respiratory Review : An... Dec 2021Thymic tumours are rare thoracic malignancies, that may be aggressive and difficult to treat. The pillars of the management include pathological review, consideration of... (Review)
Review
Thymic tumours are rare thoracic malignancies, that may be aggressive and difficult to treat. The pillars of the management include pathological review, consideration of differential diagnoses, staging and multidisciplinary discussion. Assessment of resectability is key to drive the treatment sequencing. Association with autoimmune diseases, especially myasthenia gravis, is observed, which impacts the oncological management. Networks are being built at the national and international levels. This article provides an overview of the most recent findings in the diagnosis, staging, histology, and management strategies of thymic tumours.
Topics: Diagnosis, Differential; Humans; Myasthenia Gravis; Retrospective Studies; Thymoma; Thymus Neoplasms
PubMed: 34670805
DOI: 10.1183/16000617.0394-2020 -
Chinese Medical Journal Jul 2017Good's syndrome (GS) is a rare disease characterized by thymoma, hypogammaglobulinemia, low or absent B-cells, decreased T-cells, an inverted CD4+/CD8+ T-cell ratio and... (Review)
Review
BACKGROUND
Good's syndrome (GS) is a rare disease characterized by thymoma, hypogammaglobulinemia, low or absent B-cells, decreased T-cells, an inverted CD4+/CD8+ T-cell ratio and reduced T-cell mitogen proliferative responses. GS is difficult to diagnose preoperatively due to its rarity and lack of typical symptoms, the characteristics of Chinese GS patients are still lacking. This study aimed to systematically review all the clinical, laboratory, and immunologic findings of reported cases of Chinese patients with GS.
METHODS
We searched for case reports and articles up to January 2017 using PubMed, China National Knowledge Infrastructure, Wangfang database and China Science and Technology Journal Database with the following words in combinations as key words: "thymoma," "hypogammaglobulinemia," and "Good's syndrome." The text words and MeSH terms were entered depending on the databases characteristics. The reference lists from retrieved articles were also screened for additional applicable studies. The authors were restricted to Chinese. There was no language restriction.
RESULTS
Forty-seven patients were reported in 27 studies. We found that GS has a nationwide distribution and that most cases (83%) have been described on the mainland of China. The initial clinical presentation is varied, ranging from symptoms related to the thymoma to infections resulting from immunodeficiency. Type AB (50%) is the most common histologic type of thymomas in Chinese GS patients according to the World Health Organization classification of thymomas. With respect to infection, sinopulmonary infection (74%) is the most common type, followed by skin infection (10%) and intestinal tract infection (10%). Diarrhea was presented in 36% of patients, and autoimmune manifestations were presented in 36% of patients.
CONCLUSIONS
GS is a rare association of thymoma and immunodeficiency with a poor prognosis. Astute clinical acumen and increased awareness of the clinical and immunological profile of GS are needed to increase early diagnosis, that would benefit improved therapeutic effects.
Topics: Agammaglobulinemia; Animals; China; Humans; Immunologic Deficiency Syndromes; Rare Diseases; Thymoma; Thymus Neoplasms
PubMed: 28639577
DOI: 10.4103/0366-6999.208234 -
CNS Neuroscience & Therapeutics May 2023Autoimmune encephalitis (AE) is a heterogeneous group of inflammatory central nervous system disorders caused by a misdirected immune response against self-antigens... (Review)
Review
INTRODUCTION
Autoimmune encephalitis (AE) is a heterogeneous group of inflammatory central nervous system disorders caused by a misdirected immune response against self-antigens expressed in the central nervous system. The thymus is a central organ in the immune system and thymic tumors are thought to be possible initiators of many neurological disorders. Recently, there is growing evidence that thymomas are associated with autoimmune encephalitis.
AIMS
Our study initially explored the characteristics of patients with autoimmune encephalitis combined with thymoma.
METHODS
We used patient data from January 1, 2011 to October 1, 2021 from the PubMed, Web of Science, Ovid, and CNKI platforms to analyze overall demographics, frequency of symptoms and associations, and treatment prognosis outcomes.
RESULTS
A total of 68 patients were included. There were 39 female cases (57.4%). The mean age was 50 years (IQR 40-66 years). All had acute and subacute onset. The clinical manifestations were mostly cognitive changes (70.6%), mental disorders (57.4%), and epilepsy (50.0%). The most common neuronal antibody was alpha-amino-3-hydroxy-5-methyl-4-isoxazolepropionic acid (AMPA). Magnetic resonance imaging (MRI) abnormalities were present in 81.0% of patients, mostly in the hippocampus, temporal lobe, and some in cortical and subcortical areas. Abnormalities in the electroencephalogram (EEG) in 69.8% of patients. Treatment involved immunotherapy and thymoma treatment, with 79.7% of patients improving after treatment. While 20.3% of patients had a poor prognosis. Further, 14.8% of patients relapsed. Mental disorders, autonomic dysfunction, sleep disturbances, anti-Ma2, and thymoma untreated were more frequent in patients with poor prognosis.
CONCLUSION
Thymoma-associated autoimmune encephalitis is a unique disease entity. Long-term follow-up of chest CT findings is recommended for patients with autoimmune encephalitis.
Topics: Humans; Female; Middle Aged; Thymoma; Thymus Neoplasms; Prognosis; Autoimmune Diseases of the Nervous System; Autoantibodies
PubMed: 36914970
DOI: 10.1111/cns.14166 -
World Journal of Surgical Oncology Aug 2011Among anterior mediastinal lesions, thymoma is the most common. Thymomas are tumors of thymic epithelial cell origin that are distinguished by inconsistent histological... (Review)
Review
Among anterior mediastinal lesions, thymoma is the most common. Thymomas are tumors of thymic epithelial cell origin that are distinguished by inconsistent histological and biologic behavior. Chest imaging studies typically show a round or lobulated tumor in the anterior mediastinum. Calcifications in thymomas are classically punctuate or amorphous, positioned within the lesion. Chest computed tomography (CT) features suggesting higher risk thymoma consist of tumor heterogeneity, vascular involvement, lobulation, pulmonary nodules, lymphadenopathy, and pleural manifestations. Imaging findings have an imperfect ability to predict stage and prognosis for thymoma patients. Our objective is to highlight the clinical implications of thymoma calcifications on the diagnosis, clinical manifestation and prognosis. A pubmed and google search was performed using the following words: thymoma calcification, calcified thymus, mediastinal calcification, anterior mediastinal calcification, and calcified thymoma. After reviewing 370 articles, 32 eligible articles describing thymoma calcifications were found and included in this review. Although the presence of thymus calcifications was more common in patients with invasive thymomas, they were present in significant portion of non-invasive thymomas. The presence of calcifications was not a significant factor in differentiating between benign and malignant thymoma. As a result, the type, location, size or other characteristics of thymus gland calcifications were not relevant features in clinical and radiologic diagnosis of thymoma. The histopathological diagnosis is still the only possible way to confirm the neoplastic nature of thymoma. All types of thymomas should be evaluated and managed independently of the presence of calcifications.
Topics: Biopsy; Calcinosis; Diagnosis, Differential; Humans; Radionuclide Imaging; Thymoma; Thymus Gland; Thymus Neoplasms; Tomography, X-Ray Computed
PubMed: 21861913
DOI: 10.1186/1477-7819-9-95 -
Journal of Cardiothoracic Surgery Aug 2016Thymoma is a disease with malignant potential, which has a recurrence rate after complete resection ranging from 5 to 50 %. Multiple studies on the risk factors,... (Review)
Review
Thymoma is a disease with malignant potential, which has a recurrence rate after complete resection ranging from 5 to 50 %. Multiple studies on the risk factors, treatment or prognosis have been reported. Many of them are controversial, however. In this review, we summarized some accepted risk factors, means of diagnosis and different treatments of recurrent thymoma. The risk factors of recurrent thymoma haven't been well-studied, and its management remains controversial. We reviewed the literatures and found some key points which should be noticed during the surgery of initial thymoma. Although reoperation should be taken into account preferentially, multimodal treatments are also available. The prognosis are also been discussed.
Topics: Combined Modality Therapy; Humans; Neoplasm Recurrence, Local; Prognosis; Reoperation; Risk Factors; Thymectomy; Thymoma; Thymus Neoplasms
PubMed: 27580949
DOI: 10.1186/s13019-016-0533-9