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具有横纹肌样特征的肺腺癌。一组 7 例与非小细胞肺癌的罕见类型和肺泡内填塞相关。

Lung carcinoma with rhabdoid component. A series of seven cases associated with uncommon types of non-small cell lung carcinomas and alveolar entrapment.

机构信息

Pathology Department, Hospital de León, León, Spain.

出版信息

Histol Histopathol. 2010 Oct;25(10):1287-95. doi: 10.14670/HH-25.1287.

DOI:10.14670/HH-25.1287
PMID:20712013
Abstract

Rhabdoid tumor, included in the WHO classification among large cell carcinomas of the lung, is an uncommon type of lung carcinoma with poor prognosis. We report a series of 7 cases of lung carcinomas with rhabdoid component in 10% and 80% of the tumor. The associated tumor was adenocarcinoma in 3 cases--one of them with focal micropapillary pattern--large cell carcinoma in 2 cases, squamous cell carcinoma in 1 case and pleomorphic carcinoma in 1 case. Two adenocarcinomas showed a focal spindle cell component. Micropapillary and pleomorphic types had not been reported before as a component associated with rhabdoid carcinomas. All cases were positive for vimentin, and AE1/AE3 cytokeratin and 5 cases for cytokeratin 7. All cases were negative for muscle and endothelial markers and for chromogranin A. Synaptophysin was focally positive only in one case. Alveolar trapping inside the tumor was present in 3 cases--a phenomenon not well studied in lung carcinomas and also not reported in tumors with rhabdoid component. Five patients died because of the tumor within 2 to 31 months after diagnosis, one of myocardial infarction and only one is alive and disease free 123 months after the diagnosis. In summary, we describe 7 new cases of this uncommon lung tumor with aggressive clinical course, associated with infrequent histological types in nonrhabdoid component and with alveolar trapping, a nondescribed finding.

摘要

横纹肌样瘤属于肺大细胞癌 WHO 分类,是一种罕见的肺腺癌,预后较差。我们报道了 7 例横纹肌样癌病例,肿瘤中横纹肌样成分占 10%和 80%。其中 3 例与腺癌相关——1 例具有局灶性微乳头状形态、2 例与大细胞癌相关、1 例与鳞状细胞癌相关、1 例与多形性癌相关。2 例腺癌表现为局灶性梭形细胞成分。微乳头状和多形性尚未报道为横纹肌样癌的相关成分。所有病例均表达波形蛋白、AE1/AE3 细胞角蛋白,5 例表达细胞角蛋白 7。所有病例均不表达肌肉和内皮标志物及嗜铬粒素 A。突触素仅在 1 例中呈局灶阳性。3 例肿瘤内存在肺泡捕获现象——这是肺腺癌中研究较少的现象,也未在具有横纹肌样成分的肿瘤中报道过。5 例患者因肿瘤在诊断后 2 至 31 个月内死亡,1 例死于心肌梗死,仅 1 例患者在诊断后 123 个月时存活且无疾病。总之,我们描述了 7 例这种罕见的侵袭性临床病程肺肿瘤的新病例,与非横纹肌样成分中罕见的组织学类型相关,并且与肺泡捕获相关,这是一种未描述的发现。

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