To the Editor: Multiple bile duct hamartoma [von Meyenburg complex (VMC)] is a benign liver malformation that includes biliary cystic lesions with congenital hepatic fibrosis causing ductal plate malformations (1–7). It is generally asymptomatic and tends to be identified either at autopsy or during histological examinations (1). We report a very rare case of severe portal hypertension caused b...

Corresponding author : Haeryoung Kim Department of Pathology, Seoul National University Bundang Hospital, Seoul National University College of Medicine, 82 Gumi-ro 173-beon-gil, Bundang-gu, Seongnam 463-707, Korea Tel. +82 31-787-7715, Fax. +82 31-787-4012 E-mail; [email protected] Abbreviations: BilIN, biliary intraepithelial neoplasia; CD, Caroli's disease; CHF, congenital hepatic fibrosis...

© 2015 The Korean Society of Pathologists/The Korean Society for Cytopathology This is an Open Access article distributed under the terms of the Creative Commons Attribution Non-Commercial License (http://creativecommons.org/licenses/ by-nc/3.0) which permits unrestricted non-commercial use, distribution, and reproduction in any medium, provided the original work is properly cited. pISSN 2383-7...

Intrahepatic cholangiocarcinoma (iCCA) can originate from the large bile duct group (segment ducts and area ducts), small (septal interlobular terminal (bile ductules canals of Hering) intrahepatic biliary tree, which be histopathological corresponding to type iCCA, iCCA with ductal plate malformation pattern, cholangiolocarcinoma, respectively. The challenge in pathological diagnosis above sub...

BACKGROUND In adult liver, the mesenchymal cells, portal fibroblasts and vascular smooth muscle cells can transdifferentiate into myofibroblasts, and are involved in portal fibrosis. Differential expression of markers, such as alpha-smooth muscle actin (ASMA), h-caldesmon and cellular retinol-binding protein-1 allows their phenotypic discrimination. The aim of our study was to explore the pheno...

UNLABELLED BACKGROUND& AIMS: Embryonic biliary precursor cells form a periportal sheet called the ductal plate, which is progressively remodeled to generate intrahepatic bile ducts. A limited number of ductal plate cells participate in duct formation; those not involved in duct development are believed to involute by apoptosis. Moreover, cells that express the SRY-related HMG box transcription ...

Ductal plate malformations are a heterogenous group of congenital fibrocystic liver diseases resulting from insult to the ductal plate at various stages of embryogenesis. As a result various biliary malformations, cysts, hamartomas and congenital hepatic fibrosis may be seen. We present a radiological pictorial of ductal plate malformations, accurate diagnosis of which is important for clinical...

An obese 54 year old female patient with a BMI of 43kg/m2 was scheduled to undergo diagnostic laparoscopy and laparoscopic gastric bypass operation. At laparoscopy both liver lobes showed widespread small lesions involving all surface of liver, suggestive of metastatic malignant disease. Liver was normal in shape with multiple intricate white to pale yellow nodular spots of average diameter 0.3...

The longest open reading frame of PKHD1 (polycystic kidney and hepatic disease 1), the autosomal recessive polycystic kidney disease (ARPKD) gene, encodes a single-pass, integral membrane protein named polyductin or fibrocystin. A fusion protein comprising its intracellular C-terminus, FP2, was previously used to raise a polyclonal antiserum shown to detect polyductin in several human tissues, ...