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进行性先天性肝外门体分流与多灶性肝细胞癌发生的罕见关联。

Rare association of progressive congenital extrahepatic portosystemic shunt with development of multifocal hepatocellular carcinoma.

作者信息

Landau Elliot, Shilo Dan, Rolnick Blair, Neuman Jeremy

机构信息

Radiology, Staten Island University Hospital, Staten Island, NY, USA.

AUC School of Medicine, Coral Gables, FL, USA.

出版信息

BJR Case Rep. 2016 Jul 22;3(1):20150077. doi: 10.1259/bjrcr.20150077. eCollection 2017.

DOI:10.1259/bjrcr.20150077
PMID:30363309
原文链接:https://pmc.ncbi.nlm.nih.gov/articles/PMC6159295/
Abstract

The association between the Abernethy malformation, a rare vascular anomaly in which the portal blood is diverted into the systemic circulation, and development of hepatic tumours is well established. We present a case of multifocal hepatocellular carcinoma (HCC) in the presence of extrahepatic portosystemic shunt with a diminutive portal vein (Type 2 Abernethy malformation). Abdominal ultrasound performed on a 72-year-old female presenting with elevated liver function tests found a 5.6 cm right hepatic lobe mass. Subsequent CT and MRI examinations demonstrated multifocal lesions. A diminutive portal vein was present (transverse diameter of 7 mm) with a large tortuous complex shunt (maximum transverse diameter 2.0 cm) arising at the portal vein bifurcation with branches connecting to the left renal vein and inferior vena cava. Review of a CT examination performed 10 years ago demonstrated a normal-sized portal vein (transverse diameter of 1.5 cm) with a smaller calibre portosystemic shunt (maximum transverse diameter 9 mm). To our knowledge, this is one of the first reports to demonstrate the evolution of progressive portosystemic shunting and the development of HCC.

摘要

阿伯内西畸形是一种罕见的血管异常,即门静脉血分流至体循环,其与肝肿瘤发生之间的关联已得到充分证实。我们报告一例存在肝外门体分流且门静脉细小(2型阿伯内西畸形)的多灶性肝细胞癌(HCC)病例。对一名肝功能检查结果升高的72岁女性进行腹部超声检查时,发现右肝叶有一个5.6厘米的肿块。随后的CT和MRI检查显示为多灶性病变。存在一条细小的门静脉(横径7毫米),在门静脉分叉处有一个大的迂曲复杂分流(最大横径2.0厘米),分支连接到左肾静脉和下腔静脉。回顾10年前进行的CT检查显示门静脉大小正常(横径1.5厘米),有一个较小口径的门体分流(最大横径9毫米)。据我们所知,这是首批证明进行性门体分流演变及HCC发生的报告之一。

https://cdn.ncbi.nlm.nih.gov/pmc/blobs/9ffb/6159295/e2dddc060bb0/bjrcr.20150077.g003.jpg
https://cdn.ncbi.nlm.nih.gov/pmc/blobs/9ffb/6159295/be4e6b67009c/bjrcr.20150077.g001.jpg
https://cdn.ncbi.nlm.nih.gov/pmc/blobs/9ffb/6159295/32399449063d/bjrcr.20150077.g002.jpg
https://cdn.ncbi.nlm.nih.gov/pmc/blobs/9ffb/6159295/e2dddc060bb0/bjrcr.20150077.g003.jpg
https://cdn.ncbi.nlm.nih.gov/pmc/blobs/9ffb/6159295/be4e6b67009c/bjrcr.20150077.g001.jpg
https://cdn.ncbi.nlm.nih.gov/pmc/blobs/9ffb/6159295/32399449063d/bjrcr.20150077.g002.jpg
https://cdn.ncbi.nlm.nih.gov/pmc/blobs/9ffb/6159295/e2dddc060bb0/bjrcr.20150077.g003.jpg

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