• bile duct neoplasia
• marsupials
• metastasis
• neoplasia

• Cholangiocarcinoma/veterinary
• Cholangiocarcinoma/pathology
• Male
• Animals
• Bile Duct Neoplasms/pathology
• Bile Duct Neoplasms/veterinary
• Marsupialia
• Liver Neoplasms/veterinary
• Liver Neoplasms/pathology
• Fatal Outcome

• Barn owl
• Bile duct parasite
• Bird diseases
• Platynosomum
• Trematode

• Humans
• Male
• Animals
• Dicrocoeliidae
• Strigiformes
• Trematode Infections/diagnosis
• Trematode Infections/epidemiology
• Trematode Infections/veterinary
• Brazil
• Trematoda
• Mammals

• human telomerase reverse transcriptase
• oncogenesis
• pre-neoplasia
• telomerase
• telomeres

• Animals
• Bile Duct Neoplasms/genetics
• Bile Duct Neoplasms/parasitology
• Bile Ducts, Intrahepatic/metabolism
• Bile Ducts, Intrahepatic/parasitology
• Bile Ducts, Intrahepatic/pathology
• Cholangiocarcinoma/genetics
• Cholangiocarcinoma/parasitology
• Cricetinae
• Fasciola hepatica/genetics
• Fasciola hepatica/metabolism
• Fibrosis
• Granulins/metabolism
• Intercellular Signaling Peptides and Proteins
• Nitrosamines
• Opisthorchiasis/complications
• Opisthorchiasis/parasitology
• Opisthorchiasis/pathology
• Opisthorchis/genetics
• Opisthorchis/metabolism
• Persistent Infection
• RNA, Guide, CRISPR-Cas Systems

• Wellcome Trust
• R01 CA164719 NCI NIH HHS
• 107475/Z/15/Z Wellcome Trust
• Research Program, Research and Graduate Studies, Khon Kaen University
• National Cancer Institute
• National Health and Medical Research Council

• CT
• Dysplasia and carcinomatous transformation
• Gd-EOB-DTPA-enhanced MRI
• Intrahepatic cholangiocarcinoma
• Simple hepatic cyst

• Aged
• Bile Duct Neoplasms/diagnostic imaging
• Bile Duct Neoplasms/surgery
• Bile Ducts, Intrahepatic/diagnostic imaging
• Bile Ducts, Intrahepatic/pathology
• Carcinoma in Situ
• Carcinoma, Hepatocellular/pathology
• Cholangiocarcinoma/diagnostic imaging
• Cholangiocarcinoma/surgery
• Contrast Media
• Cysts/complications
• Cysts/diagnostic imaging
• Cysts/surgery
• Gadolinium DTPA
• Humans
• Liver Diseases
• Liver Neoplasms/diagnostic imaging
• Liver Neoplasms/surgery
• Magnetic Resonance Imaging/methods
• Male
• Retrospective Studies

• Children
• Computed tomography
• Liver
• Magnetic resonance imaging
• Systemic disease
• Ultrasound

• Cholangiocarcinoma
• Liver transplantation
• Neoadjuvant chemoradiotherapy
• Primary sclerosing cholangitis

• Cancer-associated fibroblasts
• Extracellular matrix
• Fibroinflammatory risk conditions
• Immune milieu
• M2 macrophages
• Morpho-molecular classification
• Periostin
• Therapeutic targeting
• Transforming growth factor-β
• Tumor reactive microenvironment

• Animals
• Bile Duct Neoplasms/immunology
• Bile Duct Neoplasms/pathology
• Cholangiocarcinoma/immunology
• Cholangiocarcinoma/pathology
• Disease Progression
• Humans
• Tumor Microenvironment

• P30 DK034989 NIDDK NIH HHS
• R01 DK101528 NIDDK NIH HHS

• Bile duct
• Chesapeake Bay
• Liver
• Myxozoa
• Neoplasia
• Polycyclic aromatic hydrocarbons
• Teleost
• White perch

• Animals
• Bass
• Bile Ducts
• Environmental Monitoring
• Fish Diseases/epidemiology
• Neoplasms/veterinary
• Parasites
• Prevalence
• Water Pollutants, Chemical

Congenital intrahepatic bile duct dilatation without fibrosis is called Caroli disease (CD), and is called Caroli syndrome (CS) when it has fibrotic and cirrhotic liver morphology. The development of intrahepatic carcinoma is described in both conditions, but the reported incidence varies extensively. Potential risk factors for the malignant transformation were not described. Furthermore, conservative or surgical treatment is performed depending on the extent of cystic malformation, hepatic dysfunction and structural hepatic changes, but little is known about which treatment should be offered to patients with CD or CS and cancer.

To further investigate the malignant transformation in these conditions.

A systematic review of the current literature until January 2019 was performed according to the Preferred Reporting Items for Systematic Reviews and Meta-Analyses statement. A search using Medline (PubMed) was performed using a combination of Medical Subject Headings terms “caroli disease”, “caroli syndrome”, “tumor”, “malignant”, and “cholangiocarcinoma”. Only human studies published in English were used for this systematic review. The following parameters were extracted from each article: year of publication, type of study, number of patients, incidence of malignant tumor, duration of symptoms, age, sex, diagnostics, identification of tumor, surgical therapy, survival and tumor recurrence.

Twelve retrospective studies reporting the courses of 561 patients (53% females) were included in this systematic review. With a mean age of 41.6 years old (range 23 to 56 years old), patients were younger than other populations undergoing liver surgery. Depending on the size of the study population the incidence of cholangiocarcinoma varied from 2.7% to 37.5% with an overall incidence of 6.6%. There were only few detailed reports about preoperative diagnostic work-up, but a multimodal work-up including ultrasound of the liver, computed tomography, magnetic resonance imaging and endoscopic retrograde cholangiopancreatography was used in most studies. Disease duration was variable with up to several years. Most patients had episodes of cholangitis, sepsis, fever or abdominal pain. Tumor detection was an incidental finding of the surgical specimen in most cases because it is currently often impossible to detect tumor manifestation during preoperative diagnostics. Liver resection or liver transplantation was performed depending on the extent of the biliary pathology and additional alterations of the liver structure or function. No postoperative adjuvant chemotherapy was reported, but chemotherapy was administered in selected cases of tumor recurrence. Overall survival rates after one year were low at 36% and a high recurrence rate of up to 75% during the observation period.

Only few retrospective studies reported a low tumor incidence. Despite the high rate of mortality and tumor recurrence, definite surgical treatment should be offered as soon as possible.

• Malignancy
• Caroli disease
• Caroli syndrome
• Tumor
• Cholangiocarcinoma
• Review

• Adult
• Bile Duct Neoplasms
• Bile Ducts, Intrahepatic
• Caroli Disease/diagnostic imaging
• Caroli Disease/epidemiology
• Caroli Disease/surgery
• Female
• Humans
• Male
• Middle Aged
• Neoplasm Recurrence, Local
• Retrospective Studies
• Young Adult

We performed gross and histological examinations of the livers of sika deer (Cervus nippon yesoensis) in Hokkaido, Japan. Out of 1,381 deer slaughtered for venison production, thickening and dilation of the large intrahepatic bile ducts and Fasciola flukes in the duct lumens were detected in 621 deer (45.0%). Furthermore, 107 non-bile lesions (75 intrahepatic and 32 capsular lesions) were detected during gross examinations. Histologically, the bile duct lesions included chronic proliferative cholangitis, papillary hyperplasia, goblet cell and pyloric gland metaplasia, and periductal fibrosis. Many of the intrahepatic non-bile duct lesions (53/75, 71%) were considered to be Fasciola fluke migration-associated lesions, including two lesion types: necrosis, hemorrhage, and eosinophilic granuloma formation (29 lesions), and lymphoid tissue formation (24 lesions). Lymphoid tissue formation was considered to result from the persistent immune responses against dead Fasciola flukes. An epidermoid liver cyst was found incidentally, which has not been reported in the veterinary literature. In summary, this study demonstrated the predominance of fascioliasis-associated lesions in sika deer livers. The gross and histological lesions caused by Fasciola flukes in sika deer were similar to fascioliasis in other animals. Moreover, we described lymphoid tissue formation as a fascioliasis-associated lesion for the first time. The fact that bile duct lesions (45.0%) had a markedly higher prevalence than fascioliasis-associated parenchymal lesions (53/1,381, 3.8%) indicated that sika deer are a permissive host for fascioliasis. Our results provide information that will aid pathological examinations of sika deer.

• Cervus nippon yesoensis
• Fasciola
• liver
• pathology
• sika deer

• Computed tomography, multidetector
• Diagnoses, computer-assisted
• Imaging, magnetic resonance
• Intrahepatic cholangiocarcinoma
• Magnetic resonance cholangiopancreatography

• Aged
• Cholangiocarcinoma/epidemiology
• Cholangiocarcinoma/pathology
• Cholangiocarcinoma/surgery
• Female
• Hepatectomy/mortality
• Humans
• Incidence
• Liver Neoplasms/epidemiology
• Liver Neoplasms/pathology
• Liver Neoplasms/surgery
• Male
• Middle Aged
• Neoplasm Staging
• Retrospective Studies
• Survival Analysis
• Treatment Outcome

• biliary cirrhosis
• biliary strictures
• endoscopic retrograde pancreatography
• portal hypertension
• primary sclerosing cholangitis

• Bile duct carcinoma
• ERK/MAPK signaling pathway
• UCA1
• miR-122

Caroli disease (CD) is a rare congenital malformation of the liver characterized by non-obstructive, segmental, cystic dilatation of the intrahepatic bile ducts (IHDs). The clinical course is usually asymptomatic for the first 5–20 years, and symptoms may seldom occur throughout the patient’s life. Bile stagnation leads to recurrent episodes of cholangitis, stone formation, or liver abscesses, and biliary cirrhosis usually occurs years later. Here we report on a 42-year-old man diagnosed with diffuse-type CD with a characteristic central dot sign, who had multiple intrahepatic and common bile duct (CBD) stones. CBD stones were treated successfully with endoscopic retrograde cholangiopancreatography (ERCP).

• Caroli disease
• Central dot sign
• Choledocholithiasis
• Endoscopic retrograde cholangiopancreatography

• Intrahepatic cholangiocarcinoma (ICC)
• epidemiology
• risk factors

• Caroli's disease
• Cholangiocarcinoma
• Liver
• Pathology

The cystic dilatation of the biliary tract is a rare disease and uncertain origin. It is recognized more frequently in children; however, its incidence comes increasing in adults, representing 20% of the cases.

To evaluate morbimortality rates, evolution and handing of patients with cystic dilatation bile ducts in adults.

Were evaluated, retrospectively, five adults who had the diagnosis of choledochal cyst and that had been submitted to some surgical procedure.

Abdominal pain was the commonest complain to all patients. Jaundice was present in 80%. Ultrasound scanning was done in all the cases as initial examination. CT scan, magnetic resonance imaging and endoscopic retrograde cholangiopancreatography were also done in some patients; however, the diagnosis was established intra-operatively in all cases. The cyst resection with reconstruction of the biliary tract was done in 60%; the cystojejunostomy in 20%; and in 20% biliary tract drainage.

Biliary tract cystic dilatation is a rare disease. However, its incidence is increasing in the adult population, so, it must be thought as differential diagnosis when facing obstructive jaundice.

• Biliary dysplasia
• Brush cytology
• Cholangiocellular adenocarcinoma
• Endoscopic retrograde cholangiography
• Positron emission tomography
• [(18)F]FDG

• Biliary
• Cholangiocarcinoma
• Cytology
• Dysplasia
• FISH
• Primary sclerosing cholangitis

This study is intended to investigate the clinicopathological features of the intraductal papillary neoplasms of the intrahepatic bile duct (IPNB), especially focused on malignant changes.

From the institutional database of liver resection cases (Asan Medical Center, University of Ulsan College of Medicine), 18 patients who met the definition of IPNB were selected. They had undergone liver resection between February 2002 and October 2006; thus, the follow-up period was more than 5 years.

Of the 18 patients, 11 patients were male. Their mean age was 61.3±6.7 years. There were no differences between the non-malignant and malignant lesions, in the comparison of the CEA levels (5.6±2.7 vs.12.6±31.1 ng/ml, p=0.439) and the CA19-9 levels (29.2±34.7 vs.31.9±30.2 ng/ml, p=0.871). The common radiologic findings were: intraductal growing mass in 10; bile duct dilatation in 6; and saccular duct dilatation in 2. Left and right hepatectomies were performed in 15 and 3, respectively. Five patients showed benign lesions of IPNB, and 13 patients revealed malignant lesions of intraductal papillary adnocarcinoma or cholangiocarcinoma. All 4 patients with benign lesions survived for a mean period of 53 months without recurrence. In 13 patients with the malignant lesions, 1-year, 3-year, and 5-year survival rates were 100%, 84.6%, and 59.2%, respectively.

We concluded that intrahepatic IPNB is a rare type of biliary neoplasm which includes a histological spectrum, ranging from benign disease to invasive malignancy. The long-term survival was anticipated after complete curative resection.

• Intraductal papillary neoplasm
• Intrahepatic duct
• Resection

• cholangiocarcinoma
• gallbladder cancer
• colorectal cancer
• hepatocellular carcinoma
• chemoprevention

• Bile Duct Neoplasms
• Bile Ducts, Intrahepatic
• Carcinoma, Hepatocellular/epidemiology
• Cholangiocarcinoma/epidemiology
• Cholangitis, Sclerosing/epidemiology
• Colorectal Neoplasms/epidemiology
• Humans
• Liver Neoplasms/epidemiology
• Population Surveillance
• Risk Factors

• T32 DK007198 NIDDK NIH HHS
• R01 DK059427 NIDDK NIH HHS
• R56 DK059427 NIDDK NIH HHS
• R01 DK056924-10 NIDDK NIH HHS
• R01 DK059427-11 NIDDK NIH HHS
• R01 DK056924 NIDDK NIH HHS

Liver fluke disease is a chronic parasitic inflammatory disease of the bile ducts. Infection occurs through ingestion of fluke-infested, fresh-water raw fish. The most well-known species that cause human infection are Clonorchis sinensis, Opisthorchis viverrini and Opisthorchis felineus. Adult flukes settle in the small intrahepatic bile ducts and then they live there for 20-30 years. The long-lived flukes cause long-lasting chronic inflammation of the bile ducts and this produces epithelial hyperplasia, periductal fibrosis and bile duct dilatation. The vast majority of patients are asymptomatic, but the patients with heavy infection suffer from lassitude and nonspecific abdominal complaints. The complications are stone formation, recurrent pyogenic cholangitis and cholangiocarcinoma. Approximately 35 million people are infected with liver flukes throughout the world and the exceptionally high incidence of cholangiocarcinoma in some endemic areas is closely related with a high prevalence of liver fluke infection. Considering the impact of this food-borne malady on public health and the severe possible clinical consequences, liver fluke infection should not be forgotten or neglected.

• Cholangiocarcinoma
• Cholangiocarinogenesis
• Clonorchiasis
• Foodborne parasite
• Liver flukes
• Opisthorchiasis

• Animals
• Bile Duct Neoplasms/chemistry
• Bile Duct Neoplasms/pathology
• Bile Ducts, Intrahepatic
• Blotting, Western
• Cholangiocarcinoma/chemistry
• Cholangiocarcinoma/pathology
• Cricetinae
• Disease Progression
• Humans
• Immunohistochemistry
• Mesocricetus
• Precancerous Conditions/chemistry
• Precancerous Conditions/pathology
• Thioredoxins/analysis
• Thioredoxins/physiology

Because intraductal papillary mucinous neoplasm of the bile duct (IPMN-B) is believed to show a better clinical course than non-papillary biliary neoplasms, it is important to make a precise diagnosis and to perform complete surgical resection.

We herein report a case of malignant IPMN-B treated by right trisectionectomy with caudate lobectomy and extrahepatic bile duct resection. Radiologic images showed marked dilatation of the left medial sectional bile duct (B4) resulting in a bulky cystic mass with multiple internal papillary projections. Duodenal endoscopic examination demonstrated very patulous ampullary orifice with mucin expulsion and endoscopic retrograde cholangiogram confirmed marked cystic dilatation of B4 with luminal filling defects. These findings suggested IPMN-B with malignancy potential. The functional volume of the left lateral section was estimated to be 45%. A planned extensive surgery was successfully performed. The remnant bile ducts were also dilated but had no macroscopic intraluminal tumorous lesion. The histopathological examination yielded the diagnosis of mucin-producing oncocytic intraductal papillary carcinoma of the bile duct with poorly differentiated carcinomas showing neuroendocrine differentiation. The tumor was 14.0 × 13.0 cm-sized and revealed no stromal invasiveness. Resection margins of the proximal bile duct and hepatic parenchyma were free of tumor cell. The patient showed no postoperative complication and was discharged on 10^th postoperative date. He has been regularly followed at outpatient department with no evidence of recurrence.

Considering a favorable prognosis of IPMN-B compared to non-papillary biliary neoplasms, this tumor can be a good indication for aggressive surgical resection regardless of its tumor size.

• Biliary intraepithelial neoplasm
• Cytokeratin
• Intraductal papillary neoplasm
• Intrahepatic bile duct
• Intrahepatic cholangiocarcinoma
• Matrix metalloproteinase
• Mucin
• Polycomb group protein