Takahashi Kazuhiro, Sasaki Ryoko, Oshiro Yukio, Fukunaga Kiyoshi, Oda Tatsuya, Ohkohchi Nobuhiro
Organ Transplantation Gastroenterological and Hepatobiliary Surgery, Division of Clinical Medicine, Faculty of Medicine, Graduate School of University of Tsukuba, Tsukuba, Japan.
Int Surg. 2012 Oct-Dec;97(4):315-20. doi: 10.9738/CC152.1.
We encountered a rare case of a well-differentiated endocrine carcinoma originating from the bile duct in association with a congenital choledochal cyst (CCC). The patient is a 28-year-old woman referred to our clinic for pruritus. Laboratory data showed mild elevation of serum hepatobiliary enzymes. Computed tomography and magnetic resonance imaging demonstrated pancreatobiliary maljunction and a Todani type IV-A CCC from the inferior bile duct to the bilateral intrahepatic bile ducts. A solid tumor was detected in the middle portion of the common bile duct. Pancreatoduodenectomy and total extrahepatic bile duct resection was performed. Based on pathologic and immunohistochemical examinations, a diagnosis of well-differentiated endocrine carcinoma was made according to the World Health Organization criteria. To our knowledge, this is the third report of a neuroendocrine tumor originating from the bile duct in association with a CCC.
我们遇到了一例罕见的起源于胆管的高分化内分泌癌，伴有先天性胆总管囊肿（CCC）。患者为一名28岁女性，因瘙痒前来我院就诊。实验室检查数据显示血清肝胆酶轻度升高。计算机断层扫描和磁共振成像显示胰胆管汇合异常以及从肝外胆管下段至双侧肝内胆管的托达尼IV-A型CCC。在胆总管中部检测到一个实性肿瘤。行胰十二指肠切除术和肝外胆管全切除术。根据病理和免疫组化检查结果，按照世界卫生组织标准诊断为高分化内分泌癌。据我们所知，这是第三例起源于胆管并伴有CCC的神经内分泌肿瘤报告。
