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Ugeskrift For Laeger Jan 2020This review summarises the diagnostics, staging and treatment of thymic epithelial tumours, of which CT is the current primary imaging. The International Association for... (Review)
Review
This review summarises the diagnostics, staging and treatment of thymic epithelial tumours, of which CT is the current primary imaging. The International Association for the Study of Lung Cancer/International Thymic Malignancy Interest Group TNM staging and the WHO histological classifications are described. Surgery done as total thymectomy with video-assisted thoracoscopic surgery in stage I and open sternotomy in larger stages is the primary treatment if possible. Presurgical tumour reduction with chemotherapy and the possibility of adjuvant radiotherapy after R+ resection is described. Radiotherapy or chemotherapy can be considered, if definite surgery is not possible. Relapse is treated after the same principles as primary disease.
Topics: Humans; Neoplasm Recurrence, Local; Neoplasm Staging; Neoplasms, Glandular and Epithelial; Retrospective Studies; Thymectomy; Thymoma; Thymus Neoplasms
PubMed: 31928621
DOI: No ID Found -
Toxicology and Applied Pharmacology May 2023Thymoma, a tumor of thymic lymphocytes or thymic epithelial cells (TECs), is a common spontaneous tumor in Wistar Han rats, especially in females with up to 18%... (Review)
Review
Thymoma, a tumor of thymic lymphocytes or thymic epithelial cells (TECs), is a common spontaneous tumor in Wistar Han rats, especially in females with up to 18% incidence in controls. In addition to sex, there are rat strain differences in background incidence of thymomas such as Sprague Dawley versus Wistar Han rats. Human thymomas are very rare and without clear differences in incidence between males and females. Immunomodulatory and anti-inflammatory pharmaceutical drug classes, including Janus kinase inhibitors, increase the incidence of benign thymoma in two-year rat carcinogenicity studies. Potential non-genotoxic mechanisms that might contribute to the pathogenesis of thymoma development in one sex (female) Wistar Han rats include: (1) hormonal differences, (2) high proliferation rate of TECs, (3) delayed physiologic thymic involution, and/or (4) significant level of immunosuppression at high doses of a pharmaceutical drug. Factors to consider in the human cancer risk assessment of pharmaceutical-induced thymoma are: the genotoxicity of the test article, sex and strain of rats, exposure safety margins, and pathophysiologic differences and similarities of thymoma between rats and humans. Totality of weight of evidence approach and available data suggest thymomas observed in carcinogenicity studies of pharmaceutical drugs are not relevant for human risk at clinically relevant therapeutic doses.
Topics: Animals; Female; Humans; Male; Rats; Rats, Sprague-Dawley; Rats, Wistar; Risk Assessment; Thymoma; Thymus Neoplasms; Anti-Inflammatory Agents; Janus Kinase Inhibitors; Immunomodulating Agents
PubMed: 36934859
DOI: 10.1016/j.taap.2023.116471 -
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 -
Thoracic Surgery Clinics Aug 2014Thymic neuroendocrine tumors are rare and account for approximately 2% to 5% of all thymic tumors. Despite the suggestion of benign behavior implied by their name,... (Review)
Review
Thymic neuroendocrine tumors are rare and account for approximately 2% to 5% of all thymic tumors. Despite the suggestion of benign behavior implied by their name, thymic carcinoids have been noted to present a more aggressive biologic behavior than their counterparts in other sites. Because of the lack of data, adequate-sized prospective trials are required for validation, and the enrollment of patients with advanced disease into available clinical trials is encouraged.
Topics: Humans; Neuroendocrine Tumors; Thymoma; Thymus Neoplasms
PubMed: 25065934
DOI: 10.1016/j.thorsurg.2014.05.007 -
Seminars in Diagnostic Pathology Mar 2022Over the years the nomenclature of thymomas has been debated regarding the best manner in which these tumors should be grouped. In every schema presented thus far, the... (Review)
Review
Over the years the nomenclature of thymomas has been debated regarding the best manner in which these tumors should be grouped. In every schema presented thus far, the main issue has been the presence or lack of lymphocytes and accordingly, the tumors have been place into a specific category. However, even though this concept applies for most of the cases, there are numerous tumors that do not necessarily fit into those categories as either the thymomas show another cellular proliferation associated with the epithelial cells or the epithelial cell themselves are arranged in a pattern that departs from the conventional features of the classic thymoma. Herein we will emphasize those features, which in some circumstances, mainly with small mediastinoscopic biopsies may pose a considerable problem in interpretation. We do consider that the most important issue is to be familiar with the different growth pattern that these tumors may show in order to avoid misdiagnosis. In addition, we consider that regardless of the growth pattern or cellular composition of the tumor, it is highly recommended that these tumors just like any other be carefully sampled and properly stage. Although we are fully aware of the different growth pattern and specific cellular details that thymoma may show, the discussion of each one of those tumors is beyond the scope of this review. Therefore, we have placed more emphasis in those, which in our judgment are more commonly encountered in the daily practice.
Topics: Biopsy; Epithelial Cells; Humans; Lymphocytes; Thymoma; Thymus Neoplasms
PubMed: 34147302
DOI: 10.1053/j.semdp.2021.06.002 -
Current Treatment Options in Oncology Jun 2019The thymus is a key organ involved in establishing central immune tolerance. Thymic epithelial tumors (TETs) include thymomas and thymic carcinomas. Thymomas, which are... (Review)
Review
The thymus is a key organ involved in establishing central immune tolerance. Thymic epithelial tumors (TETs) include thymomas and thymic carcinomas. Thymomas, which are histologically distinct from thymic carcinomas, lead to dysregulated thymopoiesis via decreased thymic epithelial expression of AIRE and MHC Class II, as well as via alterations in thymic architecture, thereby resulting in autoimmune complications that manifest as paraneoplastic disorders (PNDs). Although progress has been made in elucidating the mechanisms underlying thymoma-associated PNDs, there remains a great need to further define the underlying mechanisms and to identify additional immune biomarkers, such as novel antibodies (in "seronegative" cases) to facilitate diagnosis and monitoring of patients. In addition, a better understanding of the pathogenesis of PNDs could lead to improved treatment strategies for both thymomas and their immune complications. In advanced, refractory cases of TETs (both thymoma and thymic carcinoma), additional therapeutic approaches are needed. Immune checkpoint inhibitors have revolutionized the treatment of several malignancies and hold promise in the treatment of TETs; however, the risks for immune-related adverse events (especially for inducing PNDs as well as in the setting of pre-existing PNDs) underscore the need to optimize patient selection and improve clinical management before there can be widespread acceptance of checkpoint inhibitor therapy in patients with TETs.
Topics: Antineoplastic Agents, Immunological; Biomarkers, Tumor; Humans; Paraneoplastic Syndromes; Thymoma; Thymus Neoplasms
PubMed: 31227926
DOI: 10.1007/s11864-019-0661-2 -
Journal of Thoracic Oncology : Official... Dec 2022The pathogenesis of thymic epithelial tumors remains largely unknown. We previously identified GTF2I L424H as the most frequently recurrent mutation in thymic epithelial...
INTRODUCTION
The pathogenesis of thymic epithelial tumors remains largely unknown. We previously identified GTF2I L424H as the most frequently recurrent mutation in thymic epithelial tumors. Nevertheless, the precise role of this mutation in tumorigenesis of thymic epithelial cells is unclear.
METHODS
To investigate the role of GTF2I L424H mutation in thymic epithelial cells in vivo, we generated and characterized a mouse model in which the Gtf2i L424H mutation was conditionally knocked-in in the Foxn1+ thymic epithelial cells. Digital spatial profiling was performed on thymomas and normal thymic tissues with GeoMx-mouse whole transcriptome atlas. Immunohistochemistry staining was performed using both mouse tissues and human thymic epithelial tumors.
RESULTS
We observed that the Gtf2i mutation impairs development of the thymic medulla and maturation of medullary thymic epithelial cells in young mice and causes tumor formation in the thymus of aged mice. Cell cycle-related pathways, such as E2F targets and MYC targets, are enriched in the tumor epithelial cells. Results of gene set variation assay analysis revealed that gene signatures of cortical thymic epithelial cells and thymic epithelial progenitor cells are also enriched in the thymomas of the knock-in mice, which mirrors the human counterparts in The Cancer Genome Atlas database. Immunohistochemistry results revealed similar expression pattern of epithelial cell markers between mouse and human thymomas.
CONCLUSIONS
We have developed and characterized a novel thymoma mouse model. This study improves knowledge of the molecular drivers in thymic epithelial cells and provides a tool for further study of the biology of thymic epithelial tumors and for development of novel therapies.
Topics: Animals; Humans; Mice; Mutation; Neoplasms, Glandular and Epithelial; Thymoma; Thymus Neoplasms; Transcription Factors, TFII; Transcription Factors, TFIII
PubMed: 36049655
DOI: 10.1016/j.jtho.2022.08.008 -
Thoracic Surgery Clinics Aug 2016Thymomas are uncommon tumors that can present as locally advanced tumors in approximately 30% of the patients. Stage and complete resection are the strongest prognostic... (Review)
Review
Thymomas are uncommon tumors that can present as locally advanced tumors in approximately 30% of the patients. Stage and complete resection are the strongest prognostic factors. For locally advanced tumors, induction treatment may improve the ability to achieve a complete resection. Combination treatment with cisplatin, doxorubicin, and cyclophosphamide is the most commonly used induction regimen. Similar rates of resectability are noted with the use of induction chemotherapy and chemoradiation therapy; however, more tumor necrosis is noted with the addition of radiation.
Topics: Antineoplastic Agents; Clinical Protocols; Humans; Induction Chemotherapy; Thymoma; Thymus Neoplasms; Treatment Outcome
PubMed: 27427527
DOI: 10.1016/j.thorsurg.2016.04.011 -
Medicina (Kaunas, Lithuania) Sep 2021: Thymomas are associated with a high frequency of paraneoplastic manifestations. Paraneoplastic syndrome (PNS) with thymoma presents a challenge to clinicians because...
: Thymomas are associated with a high frequency of paraneoplastic manifestations. Paraneoplastic syndrome (PNS) with thymoma presents a challenge to clinicians because of the need to decipher the association between the presenting symptoms and the underlying tumor. The condition most commonly noted in patients with PNS with thymoma is myasthenia gravis. Other common autoimmune diseases that may present as PNS include systemic lupus erythematosus, pure red cell aplasia, and Good syndrome. Seventy-six percent of patients with PNS-associated thymoma experience resolution of PNS after curing thymoma. : A 37-year-old man with a two-month fever accompanied by polyarthritis accidently found thymoma after contrast computed tomography scans of his chest. He accepted Video assisted thoracoscopic surgery with resection of thymoma. : Fever and polyarthritis resolved after operation but recurred in five days due to cytomegalovirus viremia, which might be predisposed by previous antibiotics treatment before the diagnosis of thymoma. : Patients with a thymoma also have a high frequency of PNS, and the most frequent condition found in patients with PNS-associated thymoma is myasthenia gravis. Fever with polyarthritis has been rarely reported as a symptom of PNS-associated thymoma. Here we reported an unusual case of PNS mimicking reactive arthritis with thymoma, as diagnosed based on the patient's clinical progression, imaging examination, and laboratory tests. The patient died of his comorbidities, and his death may have been related to long-term antibiotic use and consequent intestinal dysbiosis. This challenging case may help to inform clinicians of the need for detailed work-up of fever with unknown origin in the presence of chronic polyarthritis to prevent the overdiagnosis of inflammatory arthritis or rheumatic disease and avoid further comorbidities. Detailed work-up should include the patient's history of infections, inflammation, and malignant or nonmalignant tumors.
Topics: Adult; Arthritis, Reactive; Humans; Male; Myasthenia Gravis; Neoplasm Recurrence, Local; Paraneoplastic Syndromes; Thymoma; Thymus Neoplasms
PubMed: 34577855
DOI: 10.3390/medicina57090932 -
Journal of the American Veterinary... Jul 2018
Topics: Animals; Autopsy; Cat Diseases; Cats; Diagnosis, Differential; Dyspnea; Female; Thymoma; Thymus Neoplasms
PubMed: 29963950
DOI: 10.2460/javma.253.2.173