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Japanese Association for Research of the Thymus (JART) established by emeritus professor Akira Masaoka is a cross-sectional association of physicians and researchers and have worked in clinical and basic research on thymic neoplasms and thymus-related diseases in collaboration with The Japanese Surgical Society (JSS), Japanese Association for Chest Surgery (JACS), Japanese Association for Thoracic Surgery (JATS), Japan Lung Cancer Society (JLCS), Japanese Respiratory Society (JRS) and Japanese Association for Respiratory Endoscopy (JARE). JART has performed several clinical trials, and the most important establishment of JART in recent days is the project of the nation-wide retrospective database of thymic epithelial tumors, which contributed to UICC TNM staging. Currently, JART is building the prospective database in collaboration with the Japanese Joint Committee for Lung Cancer Registry (JJCLCR).Locally advanced thymic tumor usually invades adjacent great vessels, while the optimal treatment strategy for vessels resection and prosthetic replacement is still in controversial. We hereby present our series of patients undergoing autologous pericardial angioplasty for thymic malignancies. For invasive thymic tumors involving the superior vena cava (SVC), the replacement vessel was prepared by autologous pericardium and placed between the right atrium and distal left innominate vein stump to establish a SVC bypass. Then, the distal right innominate vein and proximal SVC were blocked, and the thymic tumor and involved vessel were completely resected, followed by SVC reconstruction using pericardium. We retrospectively analyzed the clinical characteristics and short-term outcomes of six related patients with autologous pericardial angioplasty. Due to the homologous advantages of autologous pericardial transplantation, those patients didn't need to receive anticoagulant therapy during the perioperative period, so as to avoid the occurrence of hemorrhage, embolism and other graft-related complications. There were no postoperative long-term thoracic drainage (>7 days), anastomotic bleeding, reconstructed vascular stenosis, embolism or even secondary thoracotomy and other related complications occurred in this case series. The application of autologous pericardium for the replacement of mediastinal great vessels in the surgery of locally advanced thymoma is a safe and effective technique. Compared with former artificial materials, such as polytetrafluoroethylene synthetic prosthesis, autologous pericardial transplantation avoids the occurrence of high risk graft-related complications such as postoperative hemorrhage and vascular stenosis, and its clinical application prospect is worth expecting.The Korean Association for Research on the Thymus (KART) was established in January 2014 with the following members Asan Medical Center, Samsung Seoul hospital, Seoul national university hospital and Severance hospital, known to be the top four hospitals with the most amount of surgical cases on thymic epithelial tumor (TET). The aim of KART is to establish a multi-center TET database and present the clinical features and treatment outcomes in Korea. The KART database which was based on the International Thymic Malignancy Interest Group (ITMIG) database system included 1,462 cases of retrospectively collected thymoma and thymic carcinoma from Jan 2000 to Dec 2013. Masaoka-Koga stage I consisted of 34%, stage IIA 22% and stage IIB 17%. The overall 5- and 10-year survival rates were 90% and 76% respectively. As the Masaoka-Koga stage progressed, the survival rates decreased. Through the collaboration of ITIMG, 1,327 cases from KART were registered in the database of Cancer Research And Biostatistics (CRAB) in 2019. The Korean Association for Thoracic Surgical Oncology (KATSO) is the only organization in which all thoracic surgeons in South Korea are registered. Because KART was established with the purpose of collecting data on a national scale, KART is in collaboration to produce a nationwide database system with KATSO. The new database system is planning to collaborate with the ITMIG CRAB database and will adopt a TNM staging system developed in parallel to the Masaoka-Koga system. Data is planned on being collected retrospectively and prospectively and KART data will be the foundation of the retrospective data. Once the KATSO database is fully established, the national data of TET will be used to establish a guideline and standard of the treatment for TET in South Korea and KATSO will collaborate with ITMIG.While soft tissue sarcomas typically have a spindled or pleomorphic appearance, a subset of malignant soft tissue neoplasms can have a prominent epithelioid morphology. In complex anatomic sites such as the mediastinum, such tumors can often be mistaken for a carcinoma or mesothelioma. Frequent expression of cytokeratin staining can further confound the diagnostic process and familiarity with these entities can help prevent an erroneous diagnosis. Particular entities that have been reported to occur in the mediastinum with such features include dedifferentiated liposarcoma, pleomorphic liposarcoma, malignant peripheral nerve sheath tumor, synovial sarcoma, SMARCA4-deficient thoracic sarcoma, alveolar soft part sarcoma and clear cell sarcoma. Many of these tumors exhibit unique clinical, genetic, molecular or immunohistochemical features which allow for accurate characterization. For example, pleomorphic liposarcoma contains bizarre appearing lipoblasts and dedifferentiated liposarcoma exhibits MDM2 gene amplification that is typically confirmed by fluorescence in-situ hybridization. Malignant peripheral nerve sheath tumor will often arise in association with a nerve or neurofibroma. Synovial sarcoma consistently exhibits rearrangements involving the SS18 gene and SMARCA4-deficient thoracic sarcoma shows loss of SMARCA4 staining in the tumor cells. Alveolar soft part sarcoma demonstrates an ASPL-TFE3 fusion transcript. Clear cell sarcoma often shows an EWSR1-ATF1 fusion transcript. When encountering a sarcoma of the mediastinum with epithelioid features, familiarity with these and other characteristics can help insure a correct diagnosis.Mediastinum is a Pandora's Box containing many different structures that can give origin to several cancer types. Our aims are to provide a general framework to make a diagnosis of an undifferentiated pleomorphic sarcoma and to highlight relevant immunohistochemical and molecular techniques that can help in the differential diagnosis. We, therefore, provide a simple three-step algorithmic approach to diagnose pleomorphic sarcoma, emphasizing the role of clinicopathological correlations and advocating for a "relative frequency" method, especially when the material for the diagnosis is scarce, as in small biopsies. In the first place, if clinical and/or radiological features make a non-sarcoma diagnosis more likely, it should be ruled in. Next, even if no specific non-sarcomatous diagnoses are suspected, they should always be ruled out. Lastly, since many sarcomas can have a pleomorphic appearance, specific entities should also be ruled out because their identification might affect prognosis and treatment. We then cover selected immunohistochemical and molecular ancillary tests that can come at hand in the diagnosis, highlighting the pros and cons; in particular the use and the limitations of H3K27me3 immunohistochemistry, the meaning of MDM2 amplification in the mediastinum and the implication of muscle differentiation-either smooth or skeletal-in sarcomas. The main take home messages are to always rule-out more frequent lesion first and always include clinical and radiological information in the diagnostic process.In potentially resectable non-small cell lung cancer (NSCLC) accurate mediastinal staging is crucial not only to offer the optimal management but also to avoid unnecessary surgery. Mediastinal staging is generally performed by the use of imaging techniques (computed tomography and positron emission tomography). However, the accuracy of radiological imaging in mediastinal staging is suboptimal. Therefore, additional invasive mediastinal staging is frequently required to select patients who can benefit from a neoadjuvant treatment. In recent years, endobronchial ultrasound-guided transbronchial needle aspiration (EBUS-TBNA) has progressively replaced mediastinoscopy as a test for invasive mediastinal staging. The considerable potential of EBUS-TBNA as minimally invasive staging method has been understood by pulmonologists since the early 2000s but only recently by thoracic surgeons. The clinical impact of this diagnostic technology has been broadly highlighted in the literature and EBUS-TBNA is currently considered the test of first choice in preoperative nodal staging of NSCLC. We analyze the actual role of EBUS-TBNA in invasive mediastinal staging of NSCLC patients from the thoracic surgeon point of view, with particular emphasis on the performance characteristics of this endoscopic diagnostic method as well as its clinical use within the published guidelines.Lung cancer is one of the most common solid malignant disease and an accurate staging is required for planning the most appropriate treatment. Surgery or local radiotherapy are the elective treatment for localized disease, however, the presence of mediastinal malignant lymph nodes can affect the role of surgery as a first-line therapy and a chemotherapy and/or radiotherapy treatment are recommended. Currently endoscopic ultrasound (EUS) in the mediastinum has brought the important advantage of a minimal-invasive diagnostic approach for the diagnosis and staging of malignant conditions, having the possibility of sampling lesions or lymph nodes in real-time. EUS' diagnostic capabilities have increased over time and currently it represents an excellent approach for the staging of lung cancer, especially combined to endobronchial ultrasound (EBUS). Last generation EUS equipments (echoendoscopes, ultrasound processors, needles) and tools [contrast-enhanced EUS (CH-EUS), elastography] can help the endosonographer during the ultrasound evaluation and they can increase the accuracy of lung cancer staging. A systematic method of evaluation of all the visible lymph nodes stations is fundamental to complete the lung cancer staging, as well as a correct endoscopic room organization of the equipments, healthcare personnel and devices.
Multiple common variants and also rare variants in monogenic risk genes such as
and
have been reported to be associated with risk of prostate cancer (PCa); however, the clinical setting in which germline genetic testing could be used for PCa diagnosis remains obscure. Herein, we tested the clinical utility of a 16 common variant-based polygenic risk score (PRS) that has been developed previously for Japanese men and also evaluated the frequency of PCa-associated rare variants in a prospective cohort of Japanese men undergoing prostate biopsy.
A total of 1336 patients undergoing first prostate biopsy were included. PRS was calculated based on the genotype of 16 common variants, and sequencing of 8 prostate cancer-associated genes was performed by multiplex polymerase chain reaction based target sequencing. PRS was combined with clinical factors in logistic regression models to assess whether addition of PRS improves the prediction of biopsy positivity.
The top PRS decile was associated with an odds ratio of 4.
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