冯·迈恩伯格复合体、完全性导管板畸形与肝门部胆管癌：一种罕见的关联。
Von Meyenburg complex and complete ductal plate malformation along with Klatskin tumour: a rare association.
作者信息
Gupta Ashish, Pattnaik Bramhadatta, Das Ashim, Kaman Lileswar
机构信息
PGIMER, Chandigarh, India.
Department of Histopathology, PGIMER, Chandigarh, India.
出版信息
BMJ Case Rep. 2016 Apr 18;2016:10.1136/bcr-2016-215220. doi: 10.1136/bcr-2016-215220.
Von Meyenburg complexes (VMCs), or bile duct microhamartomas, are among the constellation of defects of ductal plate malformation. These present as multiple small intrahepatic cysts and are diagnosed incidentally. Association of intrahepatic VMCs with a bile duct cancer has rarely been reported. We describe a case of a 53-year-old man presenting with obstructive jaundice. Biochemistry and radiology gave a provisional diagnosis of a resectable Klatskin tumour. The patient underwent right hepatectomy with common bile duct and caudate lobe excision. The histopathological examination demonstrated intrahepatic VMCs with complete ductal malformation and malignancy at the hilum.
冯·迈恩伯格复合体（VMCs），即胆管微错构瘤，属于导管板畸形缺陷范畴。这些病变表现为多个肝内小囊肿，多为偶然发现。肝内VMCs与胆管癌的关联鲜有报道。我们描述了一例53岁男性患者，其表现为梗阻性黄疸。生化检查和影像学检查初步诊断为可切除的肝门部胆管癌。患者接受了右半肝切除术，同时切除胆总管和尾状叶。组织病理学检查显示肝内VMCs伴有完全性导管畸形以及肝门部恶性肿瘤。
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