胸腺上皮肿瘤-回顾性分析20年在葡萄牙肺病科

I. Rodrigues, L. Nascimento, T. Gomes, Sara Heleno, A. C. Pimenta, B. Conde, A. Fernandes
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摘要

胸腺上皮肿瘤(TET)是一组罕见的纵隔原发肿瘤,包括胸腺瘤和胸腺癌。目的:描述TET患者的人口学特征、临床表现、分期和生存率;将我们的结果与当前的ESMO TET指南(2015)进行比较。方法:回顾性分析1999年至2019年我院肺科诊断为胸腺肿瘤患者的病历。结果:我们确定了30例患者,其中3例因不符合TET的定义而被排除(2例为神经内分泌胸腺肿瘤,1例为胸腺脂肪瘤)。其余27例中,男性占59.3%,平均年龄64.4±2.28岁。大多数患者(85.2%)无吸烟习惯,63%无明显合并症。既往恶性肿瘤仅占11.1%。三分之一的患者存在自身免疫性疾病;其中,77.7%表现为重症肌无力,11.1%表现为红细胞发育不全、甲状腺炎、Good综合征、小脑变性。55.5%出现非特异性呼吸道症状。根据世界卫生组织的分类,最常见的组织病理学类型是B1(22.2%),其次是AB、B2和C(各占18.5%)。Masaoka-Koga分型中,29.6%的患者处于I期,29.6%的患者处于IVA期。18名患者接受了手术。5年总生存率为75%,6例出现疾病进展。讨论与结论:与指南相比,我们的样本中男性比例更高,平均年龄更高,晚期疾病发生率更高,导致5年生存率较低。
本文章由计算机程序翻译,如有差异,请以英文原文为准。
Thymic epithelial tumours – a retrospective analysis of 20 years in a Portuguese pneumology department
Introduction: Thymic Epithelial Tumours (TET) represent a group of rare primary tumours of the mediastinum, and include Thymomas and Thymic Carcinomas. Objectives: Describe demographics, clinical presentation, staging and survival of patients diagnosed with TET; compare our results with current ESMO guidelines for TET (2015). Methods: Retrospective analysis of medical records of patients diagnosed with thymic tumours, followed in our pneumology department, between 1999 and 2019. Results: We identified 30 patients, of which 3 were excluded for not meeting the definition of TET (two were neuroendocrine thymic tumours and one a thymolipoma). Of the remaining 27, 59.3% were males, mean age 64.4±2.28 years. Most patients (85.2%) had no smoking habits and 63% had no significant comorbidities. Previous malignancy was reported in only 11.1%. Auto-immune disorders were present in a third of the patients; of those, 77.7% presented with myasthenia gravis and 11.1% with one of the following: red cell aplasia, thyroiditis, Good syndrome, cerebellar degeneration. Nonspecific respiratory symptoms were present in 55.5%. The most frequent histopathological type, according to the World Health Organisation classification, was B1 (22.2%), followed by AB, B2 and C (18.5% each). Regarding the Masaoka-Koga classification, 29.6% patients were in stage I and another 29.6% in stage IVA. Eighteen patients underwent surgery. 5-year overall survival was 75%, and progression of the disease occurred in 6 cases. Discussion and Conclusions: In comparison to the guidelines, our sample had a higher proportion of males, higher mean age and higher incidence of advanced disease, which resulted in a lower 5-year survival.
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