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Common and rare carcinomas of the thymus
Virchows Archiv ( IF 3.4 ) Pub Date : 2021-01-03 , DOI: 10.1007/s00428-020-03000-6
Anja C. Roden , Malgorzata Szolkowska

Thymic carcinoma encompasses a diverse group of rare tumors that occur almost exclusively in the prevascular (anterior) mediastinum. Thymic carcinomas have a worse outcome than thymomas with a median time to death of under 3 years. These tumors lack the typical lobulation of thymomas, exhibit commonly more cytologic atypia, are associated with a desmoplastic stromal reaction, and lack thymocytes, features that distinguish them from thymomas. The most common thymic carcinoma is squamous cell carcinoma; other subtypes include mucoepidermoid carcinoma, NUT carcinoma, and adenocarcinoma, among others. Largely due to multi-institutional and global efforts and meta-analysis of case reports and series, some of the thymic carcinoma subtypes have been studied in more detail and molecular studies have also been performed. Morphology and immunophenotype for the vast majority of thymic carcinoma subtypes are similar to their counterparts in other organs. Therefore, the distinction between thymic carcinoma and metastatic disease, which is relatively common in the prevascular mediastinum, can be challenging and in general requires clinical and radiologic correlation. Although surgical resection is the treatment of choice, only 46 to 68% of patients with thymic carcinoma can undergo resection as many other tumors present at high stage with infiltration into vital neighboring organs. These patients are usually treated with chemotherapy and/or radiation. The search for better biomarkers for prognosis and treatment of thymic carcinomas is important for improved management of these patients and possible targeted therapy.



中文翻译:

胸腺的常见和罕见癌

胸腺癌涵盖了多种稀有肿瘤,几乎只发生在血管前(前)纵隔。胸腺癌的结果要比胸腺瘤差,平均死亡时间不到3年。这些肿瘤缺乏典型的胸腺瘤小叶,通常表现出更多的细胞学上的非典型性,与增生性基质反应有关,并且缺乏胸腺细胞,这是将它们与胸腺瘤区别开的特征。最常见的胸腺癌是鳞状细胞癌。其他亚型包括黏液表皮样癌,NUT癌和腺癌等。很大程度上是由于多机构和全球的努力以及对病例报告和病例系列的荟萃分析,对一些胸腺癌亚型进行了更详细的研究,并进行了分子研究。绝大多数胸腺癌亚型的形态和免疫表型与其他器官的相似。因此,胸腺癌和转移性疾病之间的区别是比较困难的,并且通常需要临床和放射学上的联系,这在血管前纵隔中相对常见。尽管手术切除是首选治疗方法,但胸腺癌患者中只有46%至68%可以接受切除,因为许多其他晚期肿瘤会浸润到重要的邻近器官中。这些患者通常接受化疗和/或放射治疗。寻找更好的生物标志物用于胸腺癌的预后和治疗对于改善这些患者的治疗和可能的靶向治疗至关重要。

更新日期:2021-01-03
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