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Journal of Thoracic Oncology : Official... Sep 2014Thymic malignancy is often associated with paraneoplastic neurological diseases (PNDs) and recognition of these disorders is important for physicians who treat these... (Review)
Review
Thymic malignancy is often associated with paraneoplastic neurological diseases (PNDs) and recognition of these disorders is important for physicians who treat these patients. The most common thymoma-associated PNDs are myasthenia gravis (MG), acquired neuromyotonia (Isaacs' syndrome), encephalitis, Morvan's syndrome, and myositis. Diagnosis of these disorders is complex but often aided by testing for specific autoantibodies, including those to the acetylcholine receptor for MG and to contactin-associated protein-like 2, protein of the voltage-gated potassium channel complex, in patients with acquired neuromyotonia, Morvan's syndrome, or encephalitis. Patients who manifest these disorders should be screened for thymoma at diagnosis, and worsening of these PNDs may be associated with recurrent thymoma. These disorders can cause profound disability but usually respond to immunotherapy, and often improve with thymoma treatment. Close cooperation among a team of specialists is required to take proper care of these patients.
Topics: Humans; Paraneoplastic Syndromes, Nervous System; Thymoma
PubMed: 25396312
DOI: 10.1097/JTO.0000000000000300 -
Cancer Apr 2023Thymic epithelial tumors (TETs) are a rare group of malignancies arising from the thymus. Surgery remains the foundation of treatment for patients with early-stage... (Review)
Review
Thymic epithelial tumors (TETs) are a rare group of malignancies arising from the thymus. Surgery remains the foundation of treatment for patients with early-stage disease. Limited treatment options are available for the treatment of unresectable, metastatic, or recurrent TETs and are associated with modest clinical efficacy. The emergence of immunotherapies in the treatment of solid tumors has generated significant interest in understanding their role in TET treatment. However, the high rates of comorbid paraneoplastic autoimmune disorders, particularly in thymoma, have tempered expectations regarding the role of immune-based therapies. Clinical studies of immune checkpoint blockade (ICB) in thymoma and thymic carcinoma have revealed higher frequencies of immune-related adverse events (IRAEs) and limited efficacy. Despite these setbacks, the growing understanding of the thymic tumor microenvironment and systemic immune system has advanced the understanding of these diseases and provided opportunities for novel immunotherapy modalities. Ongoing studies are evaluating numerous immune-based treatments in TETs with the goal of improving clinical efficacy and mitigating IRAE risk. This review will provide insight into the current understanding of the thymic immune microenvironment, outcomes of previous ICB studies, and review treatments currently being explored for the management of TET.
Topics: Humans; Thymoma; Thymus Neoplasms; Neoplasms, Glandular and Epithelial; Immunotherapy; Immune Checkpoint Inhibitors; Tumor Microenvironment
PubMed: 36808725
DOI: 10.1002/cncr.34678 -
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 -
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 -
ESMO Open Jun 2023Thymic malignancies are rare intrathoracic tumors, which may be aggressive and difficult to treat. They represent a therapeutic challenge in the advanced/metastatic...
BACKGROUND
Thymic malignancies are rare intrathoracic tumors, which may be aggressive and difficult to treat. They represent a therapeutic challenge in the advanced/metastatic setting, with limited treatment options after the failure of first-line platinum-based chemotherapy. They are frequently associated with autoimmune disorders that also impact oncological management.
MATERIALS AND METHODS
NIVOTHYM is an international, multicenter, phase II, two-cohort, single-arm trial evaluating the activity and safety of nivolumab [240 mg intravenously (i.v.) q2 weeks] alone or with ipilimumab (1 mg /kg i.v. q6 weeks) in patients with advanced/relapsed type B3 thymoma or thymic carcinoma, after exposure to platinum-based chemotherapy. The primary endpoint is progression-free survival rate at 6 months (PFSR-6) based on RECIST 1.1 as per independent radiological review.
RESULTS
From April 2018 to February 2020, 55 patients were enrolled in 15 centers from 5 countries. Ten patients (18%) had type B3 thymoma and 43 (78%) had thymic carcinoma. The majority were male (64%), and the median age was 58 years. Among the 49 eligible patients who started treatment, PFSR-6 by central review was 35% [95% confidence interval (CI) 22% to 50%]. The overall response rate and disease control rate were 12% (95% CI 5% to 25%) and 63% (95% CI 48% to 77%), respectively. Using the Kaplan-Meier method, median progression-free survival and overall survival by local assessment were 6.0 (95% CI 3.1-10.4) months and 21.3 (95% CI 11.6-not estimable) months, respectively. In the safety population of 54 patients, adverse events (AEs) of grade 1/2 were observed in 22 (41%) patients and grade 3/4 in 31 (57%) patients. Treatment-related AEs of grade 4 included one case of neutropenia, one case of immune-mediated transaminitis, and two cases of myocarditis.
CONCLUSIONS
Nivolumab monotherapy demonstrated an acceptable safety profile and objective activity, although it has been insufficient to meet its primary objective. The second cohort of NIVOTHYM is currently ongoing to assess the combination of nivolumab plus ipilimumab.
Topics: Humans; Male; Female; Middle Aged; Nivolumab; Ipilimumab; Thymoma; Thymus Neoplasms; Progression-Free Survival
PubMed: 37285717
DOI: 10.1016/j.esmoop.2023.101576 -
Journal of Cardiothoracic Surgery Dec 2022The study's goal was to investigate the percentage of anxiety and depression in Chinese thymoma patients before surgery, and also the factors that influence it.
BACKGROUND
The study's goal was to investigate the percentage of anxiety and depression in Chinese thymoma patients before surgery, and also the factors that influence it.
METHODS
The study included patients who had an anterior mediastinal mass discovered by chest CT and were scheduled for video-assisted thoracoscopic surgery. The mental health rating scales were completed by all patients before surgery. Patients were divided into two groups based on the Hospital Anxiety and Depression Scale (HADS): anxiety/depression and non-anxiety/depression. The association between thymoma clinical factors and the HADS score was studied statistically.
RESULTS
The study comprised eighty patients with thymoma. Before the operation, 22.5% (18/80) of the patients had anxiety and/or depression. The resigned coping style characteristics, along with myasthenia gravis (MG), were associated with preoperative anxiety and depression. The greater the score of the resigned dimension, the greater the risk of anxiety and depression, based on the results of logical regression analysis. Thymoma patients with myasthenia gravis have a higher risk of anxiety and depression.
CONCLUSION
Patients with myasthenia gravis and resigned coping style were found to have higher anxiety and depression before surgery for Chinese thymoma patients.
Topics: Humans; Thymoma; Thymectomy; Depression; Thymus Neoplasms; Myasthenia Gravis; Thoracic Surgery, Video-Assisted
PubMed: 36527139
DOI: 10.1186/s13019-022-02081-5 -
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 -
International Journal of Molecular... Dec 2023Angiogenesis significantly influences the carcinogenesis of thymic epithelial tumors (TET). Both thymomas and thymic carcinoma (TC) overexpress VEGF-A and VEGFR-1 and... (Review)
Review
Angiogenesis significantly influences the carcinogenesis of thymic epithelial tumors (TET). Both thymomas and thymic carcinoma (TC) overexpress VEGF-A and VEGFR-1 and -2. This review aims to provide an appraisal of the use of anti-angiogenics in the treatment of TET. The literature research identified 16 studies that were deemed eligible for further analysis. Seven studies assessed the clinical efficacy of sunitinib and five studies the use of apatinib and/or anlotinib. The multicenter Japanese phase II REMORA trial investigated the efficacy of lenvatinib, which is a multi-targeted inhibitor of VEGFR, FGFR, RET, c-Kit, and other kinases. The objective response rate was 38% (25.6-52%), which is the highest documented in TET that progressed after first-line chemotherapy. Anti-angiogenic agents may be useful in the treatment of TET, which are not amenable to curative treatment. Their toxicity profile seems to be acceptable. However, angiogenesis inhibitors do not appear to have a major influence on either thymomas or TC, although multikinase inhibitors may have some effect on TC. The current evidence suggests that the most active agent is lenvatinib, whereas sunitinib could be proposed as an acceptable second-line therapy for TC. Further research concerning the combination of immune checkpoint inhibitors with anti-angiogenic drugs is warranted.
Topics: Humans; Thymoma; Angiogenesis Inhibitors; Sunitinib; Thymus Neoplasms; Neoplasms, Glandular and Epithelial; Multicenter Studies as Topic
PubMed: 38069386
DOI: 10.3390/ijms242317065 -
Lung Cancer (Amsterdam, Netherlands) Apr 2023The aim of this study was to differentiate benign from malignant tumors in the anterior mediastinum based on computed tomography (CT) imaging characteristics, which...
OBJECTIVES
The aim of this study was to differentiate benign from malignant tumors in the anterior mediastinum based on computed tomography (CT) imaging characteristics, which could be useful in preoperative planning. Additionally, our secondary aim was to differentiate thymoma from thymic carcinoma, which could guide the use of neoadjuvant therapy.
MATERIALS AND METHODS
Patients referred for thymectomy were retrospectively selected from our database. Twenty-five conventional characteristics were evaluated by visual analysis, and 101 radiomic features were extracted from each CT. In the step of model training, we applied support vector machines to train classification models. Model performance was assessed using the area under the receiver operating curves (AUC).
RESULTS
Our final study sample comprised 239 patients, 59 (24.7 %) with benign mediastinal lesions and 180 (75.3 %) with malignant thymic tumors. Among the malignant masses, there were 140 (58.6 %) thymomas, 23 (9.6 %) thymic carcinomas, and 17 (7.1 %) non-thymic lesions. For the benign versus malignant differentiation, the model that integrated both conventional and radiomic features achieved the highest diagnostic performance (AUC = 0.715), in comparison to the conventional (AUC = 0.605) and radiomic-only (AUC = 0.678) models. Similarly, regarding thymoma versus thymic carcinoma differentiation, the model that integrated both conventional and radiomic features also achieved the highest diagnostic performance (AUC = 0.810), in comparison to the conventional (AUC = 0.558) and radiomic-only (AUC = 0.774) models.
CONCLUSION
CT-based conventional and radiomic features with machine learning analysis could be useful for predicting pathologic diagnoses of anterior mediastinal masses. The diagnostic performance was moderate for differentiating benign from malignant lesions and good for differentiating thymomas from thymic carcinomas. The best diagnostic performance was achieved when both conventional and radiomic features were integrated in the machine learning algorithms.
Topics: Humans; Thymoma; Retrospective Studies; Lung Neoplasms; Thymus Neoplasms; Tomography, X-Ray Computed
PubMed: 36871345
DOI: 10.1016/j.lungcan.2023.02.014