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原发性纵隔肿瘤:来自单一机构的1005例报告。

Primary mediastinal neoplasms: a report of 1,005 cases from a single institution.

作者信息

Szolkowska Malgorzata, Szczepulska-Wojcik Ewa, Maksymiuk Beata, Burakowska Barbara, Winiarski Sebastian, Gatarek Juliusz, Rudzinski Piotr, Orlowski Tadeusz, Langfort Renata

机构信息

Department of Pathology, The National Tuberculosis and Lung Diseases Research Institute, Warsaw, Poland.

Department of Radiology, The National Tuberculosis and Lung Diseases Research Institute, Warsaw, Poland.

出版信息

J Thorac Dis. 2019 Jun;11(6):2498-2511. doi: 10.21037/jtd.2019.05.42.

Abstract

BACKGROUND

This study was an epidemiological analysis of all primary mediastinal neoplasms (PMNs) diagnosed between 2000 and 2016 at the National Tuberculosis and Lung Diseases Research Institute, Poland.

METHODS

All patients with any mediastinal abnormality were included in the analysis. The patients' age and gender were obtained from the institutional database.

RESULTS

From a cohort of 5,108 patients, 3,691 primary mediastinal lesions were found, including 1,005 (19%) PMNs: lymphomas (533, 53% of PMNs), thymomas (215, 21%), neurogenic tumors (NTs) (100, 10%), germ cell tumors (GCTs) (62, 6%), soft tissue tumors (STTs) (47, 5%) and thymic carcinomas/thymic neuroendocrine tumors (TCs/TNETs) (37 in total, 4%). The most frequent lymphomas were classical Hodgkin lymphomas [256] and primary mediastinal large B-cell lymphomas [163]. Type AB [73] predominated in thymomas and squamous cell carcinomas [9] and carcinoids [10] in TCs/TNETs. NTs encompassed mainly schwannomas [49], ganglioneuromas [21] and neurofibromas [10]. The most frequent STTs were hemangiomas [13] and lymphangiomas [11]. Lymphomas, thymomas and NT were more often in women, TCs/TNETs in men (P<0.001). Lymphomas predominated between the 2 and 4 decade of life, NTs under the 3 decade and thymic epithelial tumors between the 6 and 8 decade (P<0.001). There was no correlation between the subtypes of thymomas and the patients' gender (P=0.389) but it was found between histology and patients' age: in patients <30 years of age type B2 and B3 thymomas and >70 years of age AB type and micronodular thymomas with lymphoid stroma (P<0.001) predominated. In the group of GCTs half of them were malignant and these were noted exclusively in men. No correlation between subtypes of NTs or TCs/TNETs and patients' age and gender was found (P>0.05).

CONCLUSIONS

PMNs are rare conditions thus awareness of basic epidemiology may be very helpful for final diagnosis.

摘要

背景

本研究是对2000年至2016年期间在波兰国家结核病和肺部疾病研究所诊断出的所有原发性纵隔肿瘤(PMN)进行的流行病学分析。

方法

所有有纵隔异常的患者均纳入分析。患者的年龄和性别从机构数据库中获取。

结果

在5108名患者队列中,发现3691例原发性纵隔病变,其中包括1005例(19%)PMN:淋巴瘤(533例,占PMN的53%)、胸腺瘤(215例,占21%)、神经源性肿瘤(NT)(100例,占10%)、生殖细胞肿瘤(GCT)(62例,占6%)、软组织肿瘤(STT)(47例,占5%)以及胸腺癌/胸腺神经内分泌肿瘤(TC/TNET)(共37例,占4%)。最常见的淋巴瘤是经典型霍奇金淋巴瘤[256例]和原发性纵隔大B细胞淋巴瘤[163例]。胸腺瘤中AB型[73例]占主导,TC/TNET中鳞状细胞癌[9例]和类癌[10例]占主导。NT主要包括神经鞘瘤[49例]、神经节神经瘤[21例]和神经纤维瘤[10例]。最常见的STT是血管瘤[13例]和淋巴管瘤[11例]。淋巴瘤、胸腺瘤和NT在女性中更为常见,TC/TNET在男性中更为常见(P<0.001)。淋巴瘤在20至40岁之间占主导,NT在30岁以下,胸腺上皮肿瘤在60至80岁之间(P<0.001)。胸腺瘤的亚型与患者性别之间无相关性(P=0.389),但在组织学与患者年龄之间发现了相关性:在年龄<30岁的患者中,B2型和B3型胸腺瘤占主导,在年龄>70岁的患者中,AB型和伴有淋巴样间质的微结节型胸腺瘤占主导(P<0.001)。在GCT组中,其中一半是恶性的,且仅在男性中发现。未发现NT或TC/TNET的亚型与患者年龄和性别之间存在相关性(P>0.05)。

结论

PMN是罕见疾病,因此了解基本流行病学情况可能对最终诊断非常有帮助。

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