IgG4-related hepatic inflammatory pseudotumor with sclerosing cholangitis: a case report and review of the literature.

IgG4-related hepatic inflammatory pseudotumor with sclerosing cholangitis: a case report and review of the literature.
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DOI:
10.4076/1757-1626-2-7029
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发表时间:
2009-06-11
期刊:
Cases journal
影响因子:
--
通讯作者:
Joh T
Joh T
中科院分区:
其他
文献类型:
--
作者:
Naitoh I;Nakazawa T;Ohara H;Ando T;Hayashi K;Tanaka H;Okumura F;Sano H;Joh T

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炎性假瘤是一种少见的良性肿块,由慢性炎细胞浸润和纤维组织增生组成。部分炎性假瘤表现为大量IgG4阳性浆细胞浸润和闭塞性静脉炎,这是自身免疫性胰腺炎的病理特征。一位77岁的日本男性因上腹痛而住进本院。肝左叶有一孤立性肿块,动态CT表现为延迟强化。内窥镜逆行胆道造影术仅显示左肝内胆管节段性狭窄。胰腺未见异常发现。患者经临床诊断为肝内胆管细胞癌,并接受手术治疗。肝脏肿块和胆管壁的组织学检查显示大量IgG4阳性浆细胞浸润并伴有闭塞性静脉炎。最终诊断为IgG4相关性肝炎性假瘤合并硬化性胆管炎。CT延迟增强是IgG4相关炎性假瘤的特征,类似于自身免疫性胰腺炎。与自身免疫性胰腺炎无关的IgG4相关性肝炎性假瘤应考虑作为肝肿瘤鉴别诊断的实体之一。CT延迟增强对诊断IgG4相关性肝炎性假瘤有一定价值。
Inflammatory pseudotumor is rare benign mass composed of chronic inflammatory cell infiltration and proliferating fibrous tissue. Some cases of inflammatory pseudotumor show abundant infiltrating IgG4-positive plasma cells and obliterative phlebitis, which are the pathologic hallmarks of autoimmune pancreatitis. A 77-year-old Japanese man was admitted to our hospital because of epigastric pain. A solitary mass with delayed enhancement by dynamic computed tomography was present in the left hepatic lobe. Endoscopic retrograde cholangiography showed only segmental stenosis of the left intrahepatic bile duct. No abnormal findings were detected in the pancreas. The patient was clinically diagnosed as having intrahepatic cholangiocarcinoma and underwent surgery. Histological examination of the hepatic mass and bile duct wall showed abundant IgG4-positive plasma cell infiltration with obliterative phlebitis. The final diagnosis was IgG4-related hepatic inflammatory pseudotumor with sclerosing cholangitis. Delayed enhancement by computed tomography is a characteristic feature of IgG4-related inflammatory pseudotumor similar to that of autoimmune pancreatitis. IgG4-related hepatic inflammatory pseudotumor unassociated with autoimmune pancreatitis should be one of the entities considered for differential diagnosis of liver tumors. Delayed enhancement on computed tomography might be useful finding for diagnosing IgG4-related hepatic inflammatory pseudotumor.