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Gabrielle Drevet,1 Stéphane Collaud,1 François Tronc,1 Nicolas Girard2,3, Jean-Michel Maury1,3–41Department of Thoracic Surgery, Lung and Heart-Lung Transplantation, Louis Pradel Hospital, Hospices Civils de Lyon, Lyon, France; 2Institute Curie, Institut du Thorax Curie Montsouris, 75248, Paris Cedex 05, France; 3National Expert Center for Thymic Malignancies, Réseau Tumeurs THYMiques et Cancer (RYTHMIC), Lyon, France; 4IVPC UMR754 INRA, Univ Lyon, Université Claude Bernard Lyon 1, EPHE, Lyon, FranceAbstract: 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 R0 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.Keywords: thymic epithelial tumors, thymomas, thymic carcinomas, surgery, radiation, chemotherapy |
