Thymic epithelial tumors (TETs) belong to orphan oncology. The incidence of TETs is about 1.3-3.2 cases per million worldwide. Following pathology, evolution and prognosis are variable. The World Health Organization classification distinguishes thymomas and thymic carcinomas. TETs are composed of thymic epithelial tumoral cells and normal lymphocytes. The mean age at diagnosis is 50-60 years-old. There are no identified risk factors. TETs are frequently associated with paraneoplastic syndromes as myasthenia gravis. The complete R surgical resection is the most significant prognosis factor on survival. In 2010, the French National Institute of Cancer labeled the RYTHMIC network as a specific tumor board including thoracic surgeons, oncologist, and radiation therapist to define standard of care for the management of TETs. The aim of the review was to update knowledge to optimize the standard of care.
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http://dx.doi.org/10.2147/CMAR.S171683 | DOI Listing |
Mediastinum
November 2024
Department of Oncology, Mayo Clinic, Rochester, MN, USA.
Background: Thymoma is a rare mediastinal neoplasm originating from thymic epithelial cells, often associated with paraneoplastic syndromes. These syndromes can manifest as a range of autoimmune disorders, including myasthenia gravis, pure red cell aplasia, and aplastic anemia. Clinical trials involving the use of immune checkpoint inhibitors (ICIs) in thymoma have been complicated by a high incidence of immune-related adverse effects (irAEs).
View Article and Find Full Text PDFMediastinum
November 2024
University of Bern, Bern, Switzerland.
Background And Objective: Thymic epithelial tumors, including thymomas and thymic carcinomas, represent the most common mediastinal tumors and account for up to 50% of all anterior mediastinal tumors. For early stages of these thymic tumors, complete resection of the entire thymus is the recommended treatment. The transition from open surgery to video-assisted thoracoscopic surgery (VATS) and recently to robotic-assisted thoracic surgery (RATS) has fundamentally altered the treatment of thymic tumors.
View Article and Find Full Text PDFMediastinum
October 2024
Department of General Thoracic Surgery, Graduate School of Medicine, Osaka University, Osaka, Japan.
Background And Objective: Thymic epithelial tumors (TETs) are the most common neoplasm of the prevascular mediastinal compartment and are characterized by their rarity and variable clinical presentation. The present study aimed to explore the current management of patients with TET with a special focus on immunotherapy for advanced disease.
Methods: Relevant studies published between 1981 and 2024 were searched in PubMed using search terms "Thymoma", "Thymic cancer", "Myasthenia gravis", "Radiation therapy", "Surgery", and "Immunotherapy".
Mediastinum
November 2024
Department of Thoracic Surgery, Nagoya University Graduate School of Medicine, Nagoya, Japan.
Thymic epithelial tumors (TETs) are rare neoplasms that include thymomas, thymic carcinomas (TCs), and thymic neuroendocrine neoplasms (TNENs). These three tumor categories differ in aggressiveness, the incidence of recurrence after resection, the pattern of recurrence, and survival outcomes. Owing to the tumor's rarity, randomized trials have not been performed in the initial treatment setting.
View Article and Find Full Text PDFFront Immunol
January 2025
Department of Hematology, Changhai Hospital, The Second Military Medical University, Shanghai, China.
Background: Chronic graft-versus-host disease (cGVHD) manifests with characteristics of autoimmune disease with organs attacked by pathogenic helper T cells. Recent studies have highlighted the role of T cells in cGVHD pathogenesis. Due to limited understanding of underlying mechanisms, preventing cGVHD after allogenic hematopoietic cell transplantation (HCT) has become a major challenge.
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