Department of Gastroenterological Surgery, Graduate School of Life Sciences, Kumamoto University, Kumamoto 8608556, Japan.
World J Gastroenterol. 2020 May 14;26(18):2268-2275. doi: 10.3748/wjg.v26.i18.2268.
An ectopic hepatocellular carcinoma (EHCC) arises from the ectopic liver which is defined as a hepatic organ or tissue not connected to surrounding tissues. EHCC is a rare disease and it is difficult to diagnose preoperatively. Furthermore, the clinical features are not fully elucidated.
A retroperitoneal tumor (6 cm) was located at the dorsal side of the pancreas head on abdominal ultrasonography in an 81-year old woman positive for hepatitis C virus antibody. Contrast enhanced-computed tomography and gadolinium-ethoxybenzyl-diethylenetriamine pentaacetic acid-enhanced magnetic resonance imaging showed viable HCC patterns with early enhancement and delayed washout. The tumor markers - serum alpha-fetoprotein and alpha-fetoprotein-L3% - were increased to 30.1 ng/mL and 83.1%, respectively. Protein induced by vitamin K absence or antagonist-II was within normal levels (17 mAU/mL). Positron emission tomography-computed tomography showed strong accumulation into the tumor (Standardized Uptake Value max: 13.8), and the tumor cytology following endoscopic ultrasound-guided fine needle aspiration showed poorly differentiated carcinoma. Tumor extirpation was performed, and operative findings showed that the retroperitoneal tumor was disconnected from the pancreas and the liver. Swollen lymph nodes near the tumor were histologically normal. On histological examination, the tumor was finally diagnosed as EHCC with Arginase-1 positive expression.
We report our experience of a rare EHCC which was difficult to diagnose, and we present a review of the literature.
异位肝细胞癌(EHCC)源自异位肝,即与周围组织无连接的肝器官或组织。EHCC 是一种罕见疾病,术前难以诊断。此外,其临床特征尚未完全阐明。
一名 81 岁女性丙型肝炎病毒抗体阳性,腹部超声检查发现胰头背侧有一腹膜后肿瘤(6cm)。增强型计算机断层扫描和钆乙氧基苯甲基二乙三胺五乙酸增强磁共振成像显示活的 HCC 模式,表现为早期强化和延迟洗脱。肿瘤标志物——血清甲胎蛋白和甲胎蛋白-L3%分别升高至 30.1ng/ml 和 83.1%。维生素 K 拮抗剂-II 诱导蛋白水平正常(17mAU/ml)。正电子发射断层扫描-计算机断层扫描显示肿瘤强烈积聚(标准摄取值最大值:13.8),内镜超声引导下细针抽吸的肿瘤细胞学显示为低分化癌。进行了肿瘤切除术,手术发现腹膜后肿瘤与胰腺和肝脏无连接。肿瘤附近肿大的淋巴结组织学正常。组织学检查最终诊断为 EHCC,Arg-1 阳性表达。
我们报告了一例罕见的 EHCC 病例,其诊断困难,并对文献进行了回顾。