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原发性纵隔尤因肉瘤伴胸腔积液:1例报告及文献复习

Primary mediastinum Ewing's sarcoma with pleural effusion: A case report and literature review.

作者信息

Li Xuhong, Qi Shan, Zhu Taiwen, Jiang Ying, Wang Wei

机构信息

Department of Respiratory and Critical Care Medicine, Zhongnan Hospital of Wuhan University, Wuhan 430071, China.

出版信息

Open Life Sci. 2023 Aug 1;18(1):20220669. doi: 10.1515/biol-2022-0669. eCollection 2023.

DOI:10.1515/biol-2022-0669
PMID:37554964
原文链接:https://pmc.ncbi.nlm.nih.gov/articles/PMC10404896/
Abstract

Ewing's sarcoma (ES) is an aggressive cancer in young adults. Primary ES occurring in the chest with pleural effusion is even rarer. We report the case of a 15-year-old girl who presented with intermittent chest pain occurring for more than 2 months and cough and wheezing for 10 days. Radiological imaging showed a large soft tissue mass with multiple small vessel shadows near the left mediastinum and bloody pleural effusion in the left thorax. ES was diagnosed by positive immunostaining for CD99, FLI-1, and NKX2 combined with fluorescence hybridization detection of the EWSR1 gene arrangement. With chemotherapy, lung computed tomography revealed that the tumor had become much smaller, and the fluid was absorbed. We report a case of extraskeletal Ewing's sarcoma (EES) in the mediastinum with pleural effusion, which is unusual and challenging. EES is a highly malignant tumor with a poor prognosis. Early diagnosis and treatment can improve the survival rate of patients.

摘要

尤因肉瘤(ES)是一种发生于年轻成年人的侵袭性癌症。原发于胸部且伴有胸腔积液的ES更为罕见。我们报告了一例15岁女孩的病例,该女孩出现间歇性胸痛2个多月,咳嗽和喘息10天。影像学检查显示左纵隔附近有一个伴有多个小血管影的大软组织肿块,左胸腔有血性胸腔积液。通过CD99、FLI-1和NKX2免疫染色阳性,并结合EWSR1基因重排的荧光杂交检测,诊断为ES。经过化疗,肺部计算机断层扫描显示肿瘤明显缩小,积液被吸收。我们报告了一例纵隔内伴有胸腔积液的骨外尤因肉瘤(EES)病例,这种情况不常见且具有挑战性。EES是一种高度恶性肿瘤,预后较差。早期诊断和治疗可提高患者的生存率。

https://cdn.ncbi.nlm.nih.gov/pmc/blobs/be05/10404896/3a3af695c480/j_biol-2022-0669-fig003.jpg
https://cdn.ncbi.nlm.nih.gov/pmc/blobs/be05/10404896/ca83d142cad4/j_biol-2022-0669-ga001.jpg
https://cdn.ncbi.nlm.nih.gov/pmc/blobs/be05/10404896/ec9bb1d8fb4c/j_biol-2022-0669-fig001.jpg
https://cdn.ncbi.nlm.nih.gov/pmc/blobs/be05/10404896/bb5c99b6da76/j_biol-2022-0669-fig002.jpg
https://cdn.ncbi.nlm.nih.gov/pmc/blobs/be05/10404896/3a3af695c480/j_biol-2022-0669-fig003.jpg
https://cdn.ncbi.nlm.nih.gov/pmc/blobs/be05/10404896/ca83d142cad4/j_biol-2022-0669-ga001.jpg
https://cdn.ncbi.nlm.nih.gov/pmc/blobs/be05/10404896/ec9bb1d8fb4c/j_biol-2022-0669-fig001.jpg
https://cdn.ncbi.nlm.nih.gov/pmc/blobs/be05/10404896/bb5c99b6da76/j_biol-2022-0669-fig002.jpg
https://cdn.ncbi.nlm.nih.gov/pmc/blobs/be05/10404896/3a3af695c480/j_biol-2022-0669-fig003.jpg

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Regulation of EWSR1-FLI1 Function by Post-Transcriptional and Post-Translational Modifications.转录后和翻译后修饰对EWSR1-FLI1功能的调控
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Ewing Sarcoma of the Lung: Imaging of a Rare Tumor.肺尤因肉瘤:一种罕见肿瘤的影像学表现
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