Introduction: We report a case of intrahepatic cholangiocarcinoma and portal hypertension developing in a liver with biliary microhamartomas (von Meyenburg’s complex). Case Report: The patient was a 55-year-old man who had a past medical history of diffuse multiple liver abscesses. During follow-up examination, a hypovascular nodule measuring 2.1 cm in diameter was incidentally found in segment 8 of the liver. Surgical resection was performed based on a suspected diagnosis of hepatocellular carcinoma. A gastrofiberscopy examination detected characteristic findings of portal hypertensive gastropathy. During the laparotomy, multiple tiny cystic lesions were observed in a diffuse pattern across the liver surface. The liver parenchyma was slightly fibrotic and haemorrhagic. A histopathological examination revealed intrahepatic cholangiocarcinoma with vascular invasions in von Meyenburg’s complex. Multiple biliary adenomas were also observed among the biliary microhamartomas adjacent to the main tumour, suggesting that the malignant transformation of the biliary adenomas might have been responsible for the development of the intrahepatic cholangiocarcinoma. The histopathologic examination also revealed sinusoidal dilation and abnormal spacing of the portal tracts and central veins as evidence of portal hypertension.
intrahepatic cholangiocarcinoma ; portal hypertension

Objective: To examine the significance and prognostic value of the classification of hilar cholangiocarcinoma based on actual anatomical location. Methods: A retrospective study was conducted including 120 patients of hilar cholangiocarcinoma treated at the Second Affiliated Hospital,Zhejiang University School of Medicine and Xinhua Hospital Affiliated to Shanghai Jiao Tong University School of Medicine from January 2019 to December 2021. Patients with hilar cholangiocarcinoma were classified for seven types according to the site of tumor location. The clinicopathological and prognostic data of 120 patients were retrospectively analyzed(There were 57 males and 63 females,the age (M(IQR)) was 61(22)years(range:42 to 85 years)). All patients received radical resection without visible intraoperative tumor residue and negative bile duct resection margin according to intraoperative pathological biopsy. The classification variables were analyzed by Pearson χ² test or Fisher's exact probability test,one-way ANOVA or Kruskal-Wallis rank sum test.Kaplan-Meier method was used for survival analysis. Cox proportional risk model was used for prognostic factors. Results: The coincidence rate of preoperative surgical planning and actual operational styles was verified in 33 cases. Twenty-six cases were consistent,and 7 cases were inconsistent,with a coincidence rate of 78.8%. According to the actual anatomical location,patients in type of secondary branch experienced a significantly longer operation duration,a higher portal vein resection rate,margin positive rate and more advanced T stage(all P<0.05). The median overall survival time of the unilateral main trunck group was 27.0 months,and the bilateral group was 17.0 months. Survival analysis based on the tumor classification of the actual anatomical location showed that the unilateral or main trunck group predicted less aggressive clinical features and favorable outcomes(HR=1.931,95%CI:1.066 to 3.499,P<0.05). Multivariate analysis demonstrated that the actual anatomical location of the tumor type(HR=2.269,95%CI:1.333 to 3.861,P=0.003),combined liver resection(HR=0.464,95%CI:0.253 to 0.848,P=0.013) and N stage(HR=6.317,95%CI:3.083 to 12.944,P<0.01) were independent factors affecting the prognosis of patients. Conclusion: The classification based on the actual anatomy can be used as a promising scheme in refining patient stratification and predicting survival in hilar cholangiocarcinoma,and it can guide the selection of surgical methods,and predict operative safety and radical resection rate.
Bile Duct Neoplasms/surgery* ; Bile Ducts, Intrahepatic/pathology* ; China ; Cholangiocarcinoma/surgery* ; Female ; Humans ; Klatskin Tumor/surgery* ; Male ; Retrospective Studies

PURPOSE: Various treatments have recently been applied for hepatolithiasis and their results also have been reported. We introduce herein a new surgical technique, the Ventral Hilar Exposure Method, for hepatolithiasis. This method has been performed for the last ten years at our hospital, and we now report the results of this procedure. METHODS: We evaluated the outcomes of 128 patients among 153 patients who had received hepatectomy with the method of ventral hilar exposure for hepatolithiasis at our hospital from June 1994 to June 2004. We analyzed the rates of residual and recurrent stone, the risk of the treatment and the rate of concomitant cholangiocarcinoma. With these results, we evaluated the utility of the ventral hilar exposure method for hepatolithiasis. RESULTS: There was no post-operative mortality and severe complications in the 128 patients who had received hepatectomy by the ventral hilar exposure method. Among those patients, the rate of residual stone and recurrent stone were 5.4% and 4.2%, respectively. The rate of concomitant cholangiocarcinoma was 11%. CONCLUSION: The ventral hilar exposure method hepatectomy is safe and it shows a more improved treatment result for hepatolithiasis. We think that the direct exploration of intra-hepatic hilar bile duct structure during hepatectomy resulted in the improved outcomes. Therefore, we recommend this procedure of hepatectomy as a standardized surgical treatment method for hepatolithiasis.
Bile Ducts ; Bile Ducts, Intrahepatic ; Cholangiocarcinoma ; Hepatectomy ; Humans ; Mortality ; Recurrence

BACKGROUND: The relationship between hepatoliths and cholangiocarcinoma is etiologically unclear. However, histogenetic sequencing with hyperplasia, dysplasia and carcinoma can occur in the bile ducts of hepatolithiasis. METHODS: We studied 55 cases of hepatolithiasis and examined the specimens of resected liver tissue with a microscope. The growth patterns of bile duct epithelium were divided into four types: flat, tufting, micropapillary and papillary. The dysplasia was also divided into low-grade dysplasia (LGD) and high-grade dysplasia (HGD). RESULTS: Of 55 cases of hepatolithiasis, 30 cases (54.6%) were of the flat pattern, 13 cases (23.6%) the micropapillary pattern, and 11 cases (20%) the tufting pattern. Epithelial hyperplasia was noted in only 36 cases (65.5%) in the large bile ducts, but dysplastic changes were found in 19 cases. Of 19 cases of dysplasia, LGD was present in 14 cases (25.5% of total 55 cases) an HGD in 5 cases (9% of total 55 cases). The epithelial hyperplasia showed histologic growth of the flat pattern in 29 cases out of 36 cases. But LGD (14 cases) had 6 cases of the tufting pattern and 7 cases of the micropapillary pattern. HGD (5 cases) revealed 4 cases of the micropapillary pattern with one case of the tufting pattern. CONCLUSION: This study suggests that sequences of hyperplasia, low-grade dysplasia and high-grade dysplasia can play a role in the carcinogenesis of bile duct epithelium in hepatolithiasis with the histologic pattern changing from flat to micropapillary growth.
Bile Ducts ; Bile Ducts, Intrahepatic* ; Carcinogenesis ; Cholangiocarcinoma ; Epithelium* ; Hyperplasia ; Liver*

Bile Duct Neoplasms ; surgery ; Bile Ducts, Intrahepatic ; Cholangiocarcinoma ; surgery ; Humans

Brugada syndrome can be unmasked by several conditions including a febrile state, marked leukocytosis, and electrolyte disturbances. Herein, we describe a 62-year-old man with cholangiocarcinoma in the first reported case of Brugada syndrome onset following photodynamic therapy.
Bile Duct Neoplasms/*drug therapy ; *Bile Ducts, Intrahepatic ; Brugada Syndrome/diagnosis/*etiology/therapy ; Cardiopulmonary Resuscitation ; Cholangiocarcinoma/*drug therapy ; Electrocardiography ; Fatal Outcome ; Fever/etiology ; Humans ; Klatskin's Tumor/*drug therapy ; Male ; Middle Aged ; Photochemotherapy/*adverse effects ; Predictive Value of Tests ; Treatment Outcome

Biliary strictures can be caused by various diseases. Intrahepatic duct (IHD) strictures are usually related to IHD stones and cholangitis. However, focal IHD strictures without IHD stones often create diagnostic problems. Parasitic diseases such as clonorchiasis can be a rare cause of an IHD stricture. Human clonorchiasis (Clonorchis sinensis infection) is an endemic parasitic disease in Eastern Asia, including Korea, and patients acquire the infestation by eating raw fish. On radiological examinations, clonorchiasis shows typically diffuse, minimal, or mild dilatation of the small IHD, particularly in the periphery, without dilatation of the extrahepatic duct. However, diagnosis of clonorchiasis can sometimes be difficult when radiological changes are atypical. We report a case of focal left IHD dilatation caused by clonorchiasis that was confused with a malignancy.
Bile Ducts, Intrahepatic ; Cholangiocarcinoma ; Cholangitis ; Clonorchiasis ; Constriction, Pathologic ; Dilatation ; Eating ; Far East ; Humans ; Korea ; Liver Neoplasms ; Parasitic Diseases ; Cholangiocarcinoma

Biliary strictures can be caused by various diseases. Intrahepatic duct (IHD) strictures are usually related to IHD stones and cholangitis. However, focal IHD strictures without IHD stones often create diagnostic problems. Parasitic diseases such as clonorchiasis can be a rare cause of an IHD stricture. Human clonorchiasis (Clonorchis sinensis infection) is an endemic parasitic disease in Eastern Asia, including Korea, and patients acquire the infestation by eating raw fish. On radiological examinations, clonorchiasis shows typically diffuse, minimal, or mild dilatation of the small IHD, particularly in the periphery, without dilatation of the extrahepatic duct. However, diagnosis of clonorchiasis can sometimes be difficult when radiological changes are atypical. We report a case of focal left IHD dilatation caused by clonorchiasis that was confused with a malignancy.
Bile Ducts, Intrahepatic ; Cholangiocarcinoma ; Cholangitis ; Clonorchiasis ; Constriction, Pathologic ; Dilatation ; Eating ; Far East ; Humans ; Korea ; Liver Neoplasms ; Parasitic Diseases ; Cholangiocarcinoma

Clonorchiasis is known to be closely related with the development of recurrent pyogenic cholangitis and carcinoma of the bile ducts. In order to ascertain the cholangiographic signs for recurrent pyogenic cholangitis or carcinoma of the bile ducts arising in patients with clonorchiasis. we reviewed cholangiograms in 42 patients with proven clonorchiasis. The population consisted of 29 patients with clonorchiasis alone, six patients with clonorchiasis and recurrent pyogenic cholangitis, and seven patients with clonorchiasis and carcinoma of the bile ducts. Cholangiographic abnormalities in 29 patients with clonorchiasis alone, six patients with clonorchiasis and recurrent pyogenic cholangitis, and seven patients with clonorchiasis and carcinoma of the bile ducts. Cholangiographic abnormalities in 29 patients with clonorchiasis alone were intrahepatic multiple, oval, or elliptic filling defects measuring 2-10 mm in size, representing adult flukes (n=24). The peripheral bile duct were obstructed (n=18), and the margins were ragged (n=20) and hazy (n=12) the intrahepatic bile ducts were dilated diffusely (n=27), and the dilated peripheral small tributaries gave the impression of "too many ducts appearance" (n=7) and dilatation was mid (n=17) In six patients with clonorchiasis and recurrent pyogenic cholangitis, there were filling defects of stones, and the extrahepatic ducts and larger intrahepatic ducts were predominantly dilated. In seven patients with clonorchiasis and cholangiocarcinoma all the biliary tree proximal to the tumor was markedly and diffusely dilated In the latter two groups, filling defects of flukes and associated findings were less prominent, but there was disproportionately severe dilatation of too many intrahepatic ducts. In patients with recurrent pyogenic cholangitis or cholangiocarcinoma, clonorchiasis should be considered as a underlying cause when cholangiogram shows "disproportionately" severe dilatation of too many intrahepatic ducts. intrahepatic ducts.
Adult ; Bile Ducts ; Bile Ducts, Intrahepatic ; Biliary Tract ; Cholangiocarcinoma ; Cholangitis ; Clonorchiasis* ; Dilatation ; Humans ; Trematoda

Intraductal variant of peripheral cholangiocarcinoma is extremely rare. This variant shows intraductal growth and intraluminal extension without any infiltrative growth. The mode of intraductal growth is not known. The prognosis of this variant is better than that of usual cholangiocarcinoma. We report three cases, one of which is associated with Clonorchis sinensis (CS) infection. The tumors were entirely confined within the dilated peripheral tributaries of the intrahepatic bile duct. Microscopically, the tumors were well to moderately well differentiated, with a papillary or a micropapillary growth pattern. Focal clear cytoplasmic change and mucin production were noted. The tumors showed intraductal spreading without any invasion to the liver parenchyme. Mucosal hyperplasia and dysplasia were noted in the adjacent ducts. The authors assume that intraductal cholangiocarcinoma is a distinct subtype, and persistent irritation, such as, CS infection may undergo a malignant transformation through mucosal dysplasia.
Bile Ducts, Intrahepatic ; Cholangiocarcinoma* ; Clonorchis sinensis ; Cytoplasm ; Hyperplasia ; Liver* ; Mucins ; Prognosis