: Hamartomas of the bile duct (von Meyenburg complex) are benign neoplasms of the liver, constituted histologically cystic dilatations of the bile duct, encompassed by fibrous stroma. We report a 42-year-old female pa...: Hamartomas of the bile duct (von Meyenburg complex) are benign neoplasms of the liver, constituted histologically cystic dilatations of the bile duct, encompassed by fibrous stroma. We report a 42-year-old female patient with symptomatic cholecystitis, whose gross and ultrasonic appearance suggestive of multiple liver metastases. Magnetic resonance imaging and liver biopsy are the gold standards for diagnosis of this rare hepatobiliary condition.展开更多
Von Meyenburg complexes are benign liver lesions consisting of adenomatous bile duct proliferates. We present two patients suffering from esophageal cancer accompanied by the occurrence of yon Meyenburg complexes. Pre...Von Meyenburg complexes are benign liver lesions consisting of adenomatous bile duct proliferates. We present two patients suffering from esophageal cancer accompanied by the occurrence of yon Meyenburg complexes. Preoperative computerized tomography （CT） of the liver had not shown these lesions. In one of the patients, diffuse nodular manifestation was found in both liver lobes, mimicking diffuse hepatic metastases. Intraoperative frozen section revealed the benign nature of the lesions in both cases. The patients underwent esophageal resection without complications. To the best of our knowledge, the coincidence of von Meyenburg complexes and esophageal cancer has never been reported before. This uncommon entity should be taken into consideration as a differential diagnosis of liver lesions in malignancies. It underlines the importance of intraoperative frozen section for liver lesions of unknown origin.展开更多
BACKGROUND Von Meyenburg complex(VMC)(i.e.,biliary hamartoma)is a rare congenital disorder characterized by multiple dilated cystic bile ducts,without clear trends in sex or age predominance.Due to the low number of p...BACKGROUND Von Meyenburg complex(VMC)(i.e.,biliary hamartoma)is a rare congenital disorder characterized by multiple dilated cystic bile ducts,without clear trends in sex or age predominance.Due to the low number of published cases and the lack of recognized guidelines,the management of such patients remains a clinical challenge.CASE SUMMARY We present a case of symptomatic VMC that was diagnosed after imaging and histopathological examinations.Considering the patient’s condition,a conservative treatment strategy was chosen.Instrumental,laboratory,and clinical follow-up demonstrated the stable condition of the patient receiving conservative treatment.CONCLUSION VMC is a potentially non-life threatening condition,but its recognition is crucial for the management of patients.展开更多
Von Meyenburg complexes(VMCs) are a rare type of ductal plate malformation. We herein report two Chinese families with VMCs, and the suspicious gene mutation of this disease. Proband A was a 62-year-old woman with abn...Von Meyenburg complexes(VMCs) are a rare type of ductal plate malformation. We herein report two Chinese families with VMCs, and the suspicious gene mutation of this disease. Proband A was a 62-year-old woman with abnormal echographic presentation of the liver. She received magnetic resonance imaging(MRI) examination and liver biopsy, and the results showed she had VMCs. Histologically proved hepatocellular carcinoma was found 1 year after the diagnosis of VMCs. Proband B was a 57-year-old woman with intrahepatic diffuselesions displayed by abdominal ultrasonography. Her final diagnoses were VMCs, congenital hepatic fibrosis, and hepatitis B surface e antigen-negative chronic hepatitis B after a series of examinations. Then, all the family members of both proband A and proband B were screened for VMCs by MRI or ultrasonography. The results showed that four of the 11 family members from two families, including two males and two females, were diagnosed with VMCs. DNA samples were extracted from the peripheral blood of those 11 individuals of two VMCs pedigrees and subjected to polymerase chain reaction amplification of the polycystic kidney and hepatic disease 1(PKHD1) gene. Two different mutation loci were identified. Heterozygous mutations located in exon 32(c.4280 delG, p.Gly1427 ValfsX 6) in family A and exon 28(c.3118 C>T, p.Arg1040 Ter) in family B were detected. We speculate that PKHD1 gene mutations may be responsible for the development of VMCs.展开更多
This is the first report of living donor liver transplantation(LDLT) for congenital hepatic fibrosis(CHF) using a mother's graft with von Meyenburg complex. A 6-year-old girl with CHF, who suffered from recurrent ...This is the first report of living donor liver transplantation(LDLT) for congenital hepatic fibrosis(CHF) using a mother's graft with von Meyenburg complex. A 6-year-old girl with CHF, who suffered from recurrent gastrointestinal bleeding, was referred to our hospital for liver transplantation. Her 38-year-old mother was investigated as a living donor and multiple biliary hamartoma were seen on her computed tomography and magnetic resonance imaging scan. The mother's liver function tests were normal and she did not have any organ abnormality, including polycystic kidney disease. LDLT using the left lateral segment(LLS) graft from the donor was performed. The donor LLS graft weighed 250 g; the graft recipient weight ratio was 1.19%. The operation and post-operative course of the donor were uneventful and she was discharged on post-operative day(POD) 8. The graft liver function was good, and the recipient was discharged on POD 31. LDLT using a graft with von Meyenburg complex is safe and useful. Long-term follow-up is needed with respect to graft liver function and screening malignant tumors.展开更多
Cholangiocarcinoma(CC) is primarily a malignant tumor of older adults most prevalent in Southeast Asia,where liver fluke infestation is high.However the etiology in western countries is unknown.Although the incidence ...Cholangiocarcinoma(CC) is primarily a malignant tumor of older adults most prevalent in Southeast Asia,where liver fluke infestation is high.However the etiology in western countries is unknown.Although the incidence of extrahepatic cholangiocarcinoma has remained constant,incidence of intrahepatic CC(ICC) which differs inmorphology,pathogenesis,risk factors,treatment and prognosis is increasing.While this increase is associated with hepatitis C virus infection,chronic nonalcoholic liver disease,obesity,and smoking,the pathogenesis of ICC and molecular alterations underlying the carcinogenesis are not completely elucidated.Benign biliary lesions such as biliary intraepithelial neoplasia,intraductal papillary neoplasm of the bile duct,von Meyenburg complex or bile duct hamartoma,and bile duct adenoma have been associated with ICC.For each of these entities,evidence suggests or supports a role as premalignant lesions.This article summarized the important biological significance of the precursor lesions of ICC and the molecular mechanisms that may be involved in intrahepatic cholangiocarcinogenesis.展开更多
文摘: Hamartomas of the bile duct (von Meyenburg complex) are benign neoplasms of the liver, constituted histologically cystic dilatations of the bile duct, encompassed by fibrous stroma. We report a 42-year-old female patient with symptomatic cholecystitis, whose gross and ultrasonic appearance suggestive of multiple liver metastases. Magnetic resonance imaging and liver biopsy are the gold standards for diagnosis of this rare hepatobiliary condition.
文摘Von Meyenburg complexes are benign liver lesions consisting of adenomatous bile duct proliferates. We present two patients suffering from esophageal cancer accompanied by the occurrence of yon Meyenburg complexes. Preoperative computerized tomography （CT） of the liver had not shown these lesions. In one of the patients, diffuse nodular manifestation was found in both liver lobes, mimicking diffuse hepatic metastases. Intraoperative frozen section revealed the benign nature of the lesions in both cases. The patients underwent esophageal resection without complications. To the best of our knowledge, the coincidence of von Meyenburg complexes and esophageal cancer has never been reported before. This uncommon entity should be taken into consideration as a differential diagnosis of liver lesions in malignancies. It underlines the importance of intraoperative frozen section for liver lesions of unknown origin.
文摘BACKGROUND Von Meyenburg complex(VMC)(i.e.,biliary hamartoma)is a rare congenital disorder characterized by multiple dilated cystic bile ducts,without clear trends in sex or age predominance.Due to the low number of published cases and the lack of recognized guidelines,the management of such patients remains a clinical challenge.CASE SUMMARY We present a case of symptomatic VMC that was diagnosed after imaging and histopathological examinations.Considering the patient’s condition,a conservative treatment strategy was chosen.Instrumental,laboratory,and clinical follow-up demonstrated the stable condition of the patient receiving conservative treatment.CONCLUSION VMC is a potentially non-life threatening condition,but its recognition is crucial for the management of patients.
基金Supported by Pilot Project of Fujian Science and Technology Department,No.2015Y0057Fujian Medical Innovation Project,No.2018-ZQN-54Science and Technology Project of Fujian Education Department,No.JAT160211
文摘Von Meyenburg complexes(VMCs) are a rare type of ductal plate malformation. We herein report two Chinese families with VMCs, and the suspicious gene mutation of this disease. Proband A was a 62-year-old woman with abnormal echographic presentation of the liver. She received magnetic resonance imaging(MRI) examination and liver biopsy, and the results showed she had VMCs. Histologically proved hepatocellular carcinoma was found 1 year after the diagnosis of VMCs. Proband B was a 57-year-old woman with intrahepatic diffuselesions displayed by abdominal ultrasonography. Her final diagnoses were VMCs, congenital hepatic fibrosis, and hepatitis B surface e antigen-negative chronic hepatitis B after a series of examinations. Then, all the family members of both proband A and proband B were screened for VMCs by MRI or ultrasonography. The results showed that four of the 11 family members from two families, including two males and two females, were diagnosed with VMCs. DNA samples were extracted from the peripheral blood of those 11 individuals of two VMCs pedigrees and subjected to polymerase chain reaction amplification of the polycystic kidney and hepatic disease 1(PKHD1) gene. Two different mutation loci were identified. Heterozygous mutations located in exon 32(c.4280 delG, p.Gly1427 ValfsX 6) in family A and exon 28(c.3118 C>T, p.Arg1040 Ter) in family B were detected. We speculate that PKHD1 gene mutations may be responsible for the development of VMCs.
文摘This is the first report of living donor liver transplantation(LDLT) for congenital hepatic fibrosis(CHF) using a mother's graft with von Meyenburg complex. A 6-year-old girl with CHF, who suffered from recurrent gastrointestinal bleeding, was referred to our hospital for liver transplantation. Her 38-year-old mother was investigated as a living donor and multiple biliary hamartoma were seen on her computed tomography and magnetic resonance imaging scan. The mother's liver function tests were normal and she did not have any organ abnormality, including polycystic kidney disease. LDLT using the left lateral segment(LLS) graft from the donor was performed. The donor LLS graft weighed 250 g; the graft recipient weight ratio was 1.19%. The operation and post-operative course of the donor were uneventful and she was discharged on post-operative day(POD) 8. The graft liver function was good, and the recipient was discharged on POD 31. LDLT using a graft with von Meyenburg complex is safe and useful. Long-term follow-up is needed with respect to graft liver function and screening malignant tumors.
文摘Cholangiocarcinoma(CC) is primarily a malignant tumor of older adults most prevalent in Southeast Asia,where liver fluke infestation is high.However the etiology in western countries is unknown.Although the incidence of extrahepatic cholangiocarcinoma has remained constant,incidence of intrahepatic CC(ICC) which differs inmorphology,pathogenesis,risk factors,treatment and prognosis is increasing.While this increase is associated with hepatitis C virus infection,chronic nonalcoholic liver disease,obesity,and smoking,the pathogenesis of ICC and molecular alterations underlying the carcinogenesis are not completely elucidated.Benign biliary lesions such as biliary intraepithelial neoplasia,intraductal papillary neoplasm of the bile duct,von Meyenburg complex or bile duct hamartoma,and bile duct adenoma have been associated with ICC.For each of these entities,evidence suggests or supports a role as premalignant lesions.This article summarized the important biological significance of the precursor lesions of ICC and the molecular mechanisms that may be involved in intrahepatic cholangiocarcinogenesis.
