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Mediastinum (Hong Kong, China) 2020The mediastinum is the visceral compartment of thoracic cavity divided into the superior and inferior mediastinum, further inferior compartmentalize into anterior,... (Review)
Review
The mediastinum is the visceral compartment of thoracic cavity divided into the superior and inferior mediastinum, further inferior compartmentalize into anterior, middle, and posterior mediastinum. Lymphoma in the mediastinum may be primary or secondary to systemic disease. Lymphoma may arise from lymphoid organs-like thymus, mediastinal lymph nodes or other mediastinal organs like heart, lung, pleura, and pericardium. It comprises about 12% of all the mediastinal tumors in adults however, it constitutes 50% of the pediatric mediastinal mass. Anatomically lymphoma most commonly involves anterior mediastinum. Among the pediatric mediastinal lymphomas, lymphoblastic lymphoma (LBL) predominate followed by Hodgkin lymphoma (HL), primary mediastinal large B cell lymphoma (PMBCL) and very rarely Grey zone lymphoma. Other types of non-HLs (NHLs) are rare among pediatric population. Radiologically and clinically present as an anterior mediastinal mass with symptoms of dyspnea, cough, and superior vena cava syndrome. Also, clinically and radiologically all the pediatric mediastinal mass shares the overlapping features, hence, for treatment and prognostic points of view its essential to differentiate the three entities, i.e., LBL, HL and PMBCL. The pathological diagnosis of pediatric mediastinal lymphomas is quite challenging for general histopathologists. In this review, we describe the pathology, genetics, differential diagnosis, treatment, prognosis, and a simplified histopathological and immunophenotypical approach to differentiate the pediatric mediastinal lymphomas.
PubMed: 35118290
DOI: 10.21037/med-20-37 -
Journal of Thoracic Disease Dec 2020Thymic tumours are a heterogeneous group of malignancies with a range of clinical presentations. The most common types are thymoma and thymic carcinoma, but overall it... (Review)
Review
Thymic tumours are a heterogeneous group of malignancies with a range of clinical presentations. The most common types are thymoma and thymic carcinoma, but overall it remains a rare cancer, and one without a clear aetiology. In this review of the epidemiology of the disease, the relationship between sex, age, and ethnicity is reviewed, along with limited evidence on the genetics of the condition. In terms of risk factors and potential causative factors, environmental exposures such as tobacco, radiation, alcohol, or diet, seem to be irrelevant, but there is some evidence linking the development of thymoma and thymic carcinoma with viral conditions, including Epstein Barr Virus. But data is not conclusive, and in the absence of large patient numbers, it is difficult to prove causation. There has been good research looking at the link between thymoma and other malignancies, either before or after the diagnosis. There does not appear to be a significant increased likelihood of thymoma following other malignancies. But, there is a suggestion, in several papers, that there is an increased risk of other malignancies following the diagnosis of thymoma, although the magnitude of this risk is disputed. There does appear to be an increased risk of non-Hodgkins Lymphoma after a diagnosis of thymoma, and this could be related to a disruption in T-cell function caused by either the disease process or the treatment directed at the thymoma. In summary though, it is a rare malignant process with a variety of presentations, often limited to the anterior mediastinum, and without an aggressive disease profile.
PubMed: 33447443
DOI: 10.21037/jtd-2019-thym-02 -
Turk Gogus Kalp Damar Cerrahisi Dergisi Jan 2024Mediastinal tumors are the most common thoracic tumor in the pediatric population. They include a spectrum of tumors, and most are malignant. These lesions can be... (Review)
Review
Mediastinal tumors are the most common thoracic tumor in the pediatric population. They include a spectrum of tumors, and most are malignant. These lesions can be anatomically and radiologically classified by means of compartments; anterior, middle, and posterior. Symptoms, signs, localization of the tumor, age of the child, and tumor markers are key points of diagnosis. Surgical approaches are typically needed for diagnosis, but sometimes tru-cut needle biopsies may be sufficient. Mediastinoscopy, mediastinotomy, and video-assisted thoracoscopic surgery may be used in the diagnostic workup of mediastinal tumors in children as they are used in adults. Frequently, diagnosis and treatment are both established by means of surgery. Surgery remains the mainstay of treatment of most benign and malignant nonlymphoid tumors. Combined modality of treatment incorporating chemotherapy and radiotherapy is often required in malignant tumors and is associated with high survival rates in these patients.
PubMed: 38584788
DOI: 10.5606/tgkdc.dergisi.2024.25799 -
Diagnostics (Basel, Switzerland) Sep 2023Thymomas are considered one of the most prevalent types of mediastinal epithelial tumors, which frequently develop in the anterior mediastinum. Due to their rarity,... (Review)
Review
Thymomas are considered one of the most prevalent types of mediastinal epithelial tumors, which frequently develop in the anterior mediastinum. Due to their rarity, these tumors' nomenclature, classification, and staging are likely to be the subject of debate and argument for most expert pathologists. Furthermore, the significance of thymoma histologic classifications have been debated over the past twenty years. While certain advocates argue that staging at the time of diagnosis is more significant, others believe that histologic subtyping has a significant impact on how patients behave clinically. In this review, we will focus on some of the challenges that diagnostic surgical pathologists may experience while evaluating the histopathology of thymomas and staging these tumors. We will additionally glance over the clinical characteristics of these distinct tumors and the current management strategy.
PubMed: 37761349
DOI: 10.3390/diagnostics13182982