Drevet Gabrielle, Collaud Stéphane, Tronc François, Girard Nicolas, Maury Jean-Michel
Department of Thoracic Surgery, Lung and Heart-Lung Transplantation, Louis Pradel Hospital, Hospices Civils de Lyon, Lyon, France.
Institute Curie, Institut du Thorax Curie Montsouris, 75248, Paris Cedex 05, France.
Cancer Manag Res. 2019 Jul 22;11:6803-6814. doi: 10.2147/CMAR.S171683. eCollection 2019.
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.
胸腺上皮肿瘤(TETs)属于罕见肿瘤。全球范围内,TETs的发病率约为每百万人口1.3 - 3.2例。根据病理情况,其发展和预后各不相同。世界卫生组织分类将胸腺瘤和胸腺癌区分开来。TETs由胸腺上皮肿瘤细胞和正常淋巴细胞组成。诊断时的平均年龄为50 - 60岁。目前尚未发现风险因素。TETs常与副肿瘤综合征如重症肌无力相关。完整的R手术切除是影响生存的最重要预后因素。2010年,法国国家癌症研究所将RYTHMIC网络标记为一个特定的肿瘤专家委员会,成员包括胸外科医生、肿瘤学家和放射治疗师,旨在确定TETs管理的护理标准。本综述的目的是更新知识以优化护理标准。