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以布加综合征为表现的肝内胆管癌:一例报告及文献复习

Intrahepatic cholangiocarcinoma presenting as the Budd-Chiari syndrome: a case report and literature review.

作者信息

Law Joanna K, Davis Jenny, Buckley Anne, Salh Baljinder

机构信息

Department of Medicine, Vancouver Hospital and Health Sciences Centre, British Columbia.

出版信息

Can J Gastroenterol. 2005 Dec;19(12):723-8. doi: 10.1155/2005/943269.

Abstract

Intrahepatic cholangiocarcinoma, an increasingly recognized primary tumour of the liver, is associated with a very poor prognosis. A patient with this tumour who presented with Budd-Chiari syndrome (the first to the authors' knowledge in Western literature and only the third patient overall) secondary to extensive thrombosis in his inferior vena cava extending from the right atrium down to his iliac vessels is described. Neither curative nor palliative intervention was deemed to be an option in this patient, who deteriorated rapidly while on anti-coagulants. Postmortem examination confirmed the radiological findings, and histological analysis revealed characteristic appearances of this tumour within the biliary tree and invasion into the inferior vena cava. Furthermore, biliary dysplasia, which can be a precursor to this cancer, was also noted within some of the bile ducts.

摘要

肝内胆管癌是一种越来越受到认可的原发性肝癌,其预后非常差。本文描述了一名患有肝内胆管癌的患者,该患者出现布加综合征(据作者所知,这是西方文献中的首例,也是全球第三例),继发于从右心房延伸至髂血管的下腔静脉广泛血栓形成。由于该患者在接受抗凝治疗时病情迅速恶化,因此无论是根治性治疗还是姑息性治疗都不是可行的选择。尸检证实了影像学检查结果,组织学分析显示胆管树内有该肿瘤的特征性表现,并侵犯了下腔静脉。此外,在一些胆管内还发现了可能是这种癌症前驱病变的胆管发育异常。

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