Nakanuma Y, Kurumaya H, Ohta G
Virchows Arch A Pathol Anat Histopathol. 1984;404(4):341-50. doi: 10.1007/BF00695218.
Multiple serous cysts involving only the hepatic hilum and larger portal tracts were found incidentally in 8 autopsy cases with several underlying liver diseases. The cysts were mostly small in size, and did not communicate with the lumena of the biliary tree. The lining of the cysts consisted of a single layer of columnar or cuboidal epithelium and the surrounding fibrous tissue was scanty. These were not associated with polycystic disease of the kidneys or cystic dilatation of the biliary tree. Cysts were absent in the liver parenchyma and an association with von Meyenburg complexes was rare. In the vicinity of the cysts there were many lobules of the glands which are physiologically present in the periductal connective tissue of the large intrahepatic bile ducts. It was noted that some of these glandular elements around the cysts disclosed varying degrees of cystic luminal dilatations. Almost all patients with such cysts had severe portal hypertension and over a half had occluding thromboemboli in the portal veins. These results lead us to propose the hypothesis that multiple cysts involving the hepatic hilum and the larger portal tracts are derived from the periductal glands in close association with an intrahepatic circulatory disturbance of the portal venous system.
在8例患有多种潜在肝脏疾病的尸检病例中，偶然发现多个浆液性囊肿仅累及肝门和较大的门静脉分支。囊肿大多体积较小，不与胆管树腔相通。囊肿内衬为单层柱状或立方上皮，周围纤维组织稀少。这些囊肿与肾多囊病或胆管树囊性扩张无关。肝实质内无囊肿，与梅氏复合体的关联罕见。在囊肿附近有许多腺体小叶，这些腺体在大肝内胆管的导管周围结缔组织中生理性存在。值得注意的是，囊肿周围的一些腺性成分显示出不同程度的囊性管腔扩张。几乎所有患有此类囊肿的患者都有严重的门静脉高压，超过一半的患者门静脉内有闭塞性血栓栓塞。这些结果使我们提出一个假说，即累及肝门和较大门静脉分支的多个囊肿起源于与门静脉系统肝内循环障碍密切相关的导管周围腺体。
