Thomas P A, Payan-Defais M J
Service de Chirurgie Thoracique, Hôpital Sainte-Marguerite, 270 Boulevard Sainte-Marguerite, 13274 Marseille cedex 9, France.
Rev Pneumol Clin. 2010 Feb;66(1):41-51. doi: 10.1016/j.pneumo.2009.12.004. Epub 2010 Feb 4.
Epithelial tumours of the thymus include thymomas, thymic carcinomas and neuro-endocrine tumours. Rare, they nevertheless represent 20% of all mediastinal tumours and 50% of those located in the anterior mediastinum. Thymomas, in particular, can be associated to auto-immune disorders, among which predominates myasthenia gravis. Their clinical behaviour varies widely, from a relative indolence to the potential of lymph node and/or systematic metastases. However, even patients with an invasive disease may have a long clinical history, explaining that a 10-year or 20-year survival from diagnosis does not imply a definitive cure. In daily practice, both the clinical Masaoka's staging system and the WHO histological classification condition the treatment strategies and allow to anticipate the prognosis. The initial treatment, as well as that of the recurrence, is based mainly on a complete resection. Postoperative radiotherapy is systematically added to the treatment of invasive tumours and/or to those with an aggressive histological subtype. Inoperable or metastatic tumours require a cisplatine and anthracyclin-based chemotherapy, followed by radical surgery and/or radiotherapy.
胸腺上皮性肿瘤包括胸腺瘤、胸腺癌和神经内分泌肿瘤。这些肿瘤虽罕见,但占所有纵隔肿瘤的20%,占前纵隔肿瘤的50%。特别是胸腺瘤,可与自身免疫性疾病相关,其中重症肌无力最为常见。其临床行为差异很大,从相对惰性到有潜在的淋巴结和/或全身转移。然而,即使是患有侵袭性疾病的患者也可能有较长的临床病史,这说明从诊断起存活10年或20年并不意味着彻底治愈。在日常实践中,临床马萨oka分期系统和世界卫生组织组织学分类决定了治疗策略,并有助于预测预后。初始治疗以及复发后的治疗主要基于完整切除。对于侵袭性肿瘤和/或具有侵袭性组织学亚型的肿瘤,术后放疗会系统性地加入治疗中。无法手术或转移性肿瘤需要以顺铂和蒽环类药物为基础的化疗,随后进行根治性手术和/或放疗。