Song Joon Seon, Lee Young-Joo, Kim Kyoung Won, Huh Jooryung, Jang Se Jin, Yu Eunsil
Department of Pathology, University of Ulsan College of Medicine, Asan Medical Center, Songpa-gu, Seoul, Korea.
Pathol Int. 2008 Aug;58(8):503-12. doi: 10.1111/j.1440-1827.2008.02264.x.
Although von Meyenburg complexes (VMC) are largely considered to be innocuous, neoplastic transformations have been described. The present report describes four cases of cholangiocarcinoma (CC) occurring on a background of VMC. The patients were all male and aged 69, 59, 68 and 75 years, respectively. While two patients were asymptomatic, the other two had a history of colon cancer. Radiologically the tumors measured 3, 4, 4.5 and 10 cm and were well enhanced from the arterial to delayed portal phase. Microscopically, the tumor consisted of multiple foci of characteristic VMC, and had a gradual transition from VMC to hyperplasia or dysplasia and well- to moderately differentiated adenocarcinomas. One patient had combined hepatocellular carcinoma (HCC) and CC, occurring in the high grade dysplastic nodule and VMC. On immunohistochemistry the epithelial cells of the VMC and CC were immunopositive for cytokeratin (CK) 7 in three patients, with another patient being focally positive only for CK19. The Ki-67 labeling indices increased from the VMC to the dysplastic areas and then to the carcinomas. As a potentially precancerous lesion, VMC should be carefully followed up in terms of any size increases. Thus, biopsies are essential to determine any proliferative epithelial changes including dysplasia and malignant transformation.
尽管肝内胆管错构瘤（VMC）大多被认为是无害的，但已有肿瘤转化的相关描述。本报告描述了4例发生在VMC背景下的胆管癌（CC）。患者均为男性，年龄分别为69岁、59岁、68岁和75岁。其中2例患者无症状，另外2例有结肠癌病史。影像学检查显示肿瘤大小分别为3cm、4cm、4.5cm和10cm，从动脉期到延迟门静脉期均有明显强化。显微镜下，肿瘤由多个特征性VMC病灶组成，呈现从VMC逐渐过渡到增生或发育异常以及高分化至中分化腺癌的过程。1例患者在高级别发育异常结节和VMC中合并有肝细胞癌（HCC）和CC。免疫组化结果显示，3例患者的VMC和CC上皮细胞细胞角蛋白（CK）7免疫阳性，另1例仅在局部对CK19呈阳性。Ki-67标记指数从VMC到发育异常区域再到癌呈递增趋势。作为一种潜在的癌前病变，对于VMC应密切随访其大小变化。因此，活检对于确定包括发育异常和恶性转化在内的任何增殖性上皮改变至关重要。
