BACKGROUND Intrahepatic bile duct adenoma(BDA)is one of the rarest of the rare benign tumors of the liver in the pediatric age group.It arises from the epithelial lining of intrahepatic bile ducts.The overall incidenc...BACKGROUND Intrahepatic bile duct adenoma(BDA)is one of the rarest of the rare benign tumors of the liver in the pediatric age group.It arises from the epithelial lining of intrahepatic bile ducts.The overall incidence of BDA is 1.3%of all primary benign liver tumors.Few case reports of this rare tumor occurring in adult population are present in the literature and to date,only one pediatric case has been reported worldwide.CASE SUMMARY An 18-month-old male child presented with chief complaints of mass per abdomen for 8 mo.Computerized tomography abdomen revealed a well-defined exophytic solid tumor arising from the posteroinferior margin of the right lobe of the liver with heterogenous enhancement and cystic changes,suggesting a suspicion of hepatoblastoma.Non-anatomical liver resection was done and a tumor of 10 cm×9.5 cm was excised.Histopathology of the specimen was conclusive with the diagnosis of intrahepatic bile duct adenoma,which was further supported by immunohistochemistry panel testing.The post-operative period was uneventful.On follow-up,the child was doing well and had no postoperative complication.CONCLUSION Intrahepatic bile duct adenoma in children is very rare and to date only one case has been reported.This is the second pediatric case of intrahepatic bile duct adenoma in the world.Additionally this is the first ever case of such a large tumor presenting in a child.展开更多
We report a case of a 75-year-old man with cystic micropapillary neoplasm of peribiliary glands detected preoperatively by radiologic examination. Enhanced computed tomography showed a low-density mass 2.2 cm in diame...We report a case of a 75-year-old man with cystic micropapillary neoplasm of peribiliary glands detected preoperatively by radiologic examination. Enhanced computed tomography showed a low-density mass 2.2 cm in diameter in the right hepatic hilum and a cystic lesion around the common hepatic duct. cholangiocarcinoma, right hepatectomy with caudate lobectomy and bile duct resection were performed. Pathological examination revealed perihilar cholangiocarcinoma mainly involving the right hepatic duct. The cystic lesion was multilocular and covered by columnar lining epithelia exhibiting increased proliferative activity and p53 nuclear expression; it also contained foci of micropapillary and glandular proliferation. Therefore, the lesion was diagnosed as a cystic micropapillary neoplasm of peribiliary glands and resembled flat branch-type intraductal papillary mucinous neoplasm of the pancreas. Histological examination showed the lesion was discontinuous with the perihilar cholangiocarcinoma. Immunohistochemistry showed the cystic neoplasm was strongly positive for MUC6 and that the cholangiocarcinoma was strongly positive for MUC5 AC and S100 P. These results suggest these two lesions have different origins. This case warrants further study on whether this type of neoplasm is associated with concomitant cholangiocarcinoma as observed in pancreatic intraductal papillary mucinous neoplasm with concomitant pancreatic duct adenocarcinoma.展开更多
Biliodigestive anastomosis between the extrahepatic bile duct and the intestine for bile duct disease is a gastrointestinal reconstruction that abolishes duodenal papilla function and frequently causes retrograde chol...Biliodigestive anastomosis between the extrahepatic bile duct and the intestine for bile duct disease is a gastrointestinal reconstruction that abolishes duodenal papilla function and frequently causes retrograde cholangitis.This chronic inflammation can cause liver dysfunction,liver abscess,and even bile duct cancer.Although research has been conducted for over 100 years to directly repair bile duct defects with alternatives,no bile duct substitute(BDS)has been developed.This narrative review confirms our understanding of why bile duct alternatives have not been developed and explains the clinical applicability of BDSs in the near future.We searched the PubMed electronic database to identify studies conducted to develop BDSs until December 2021 and identified studies in English.Two independent reviewers reviewed studies on large animals with 8 or more cases.Four types of BDSs prevail:Autologous tissue,non-bioabsorbable material,bioabsorbable material,and others(decellularized tissue,3D-printed structures,etc.).In most studies,BDSs failed due to obstruction of the lumen or stenosis of the anastomosis with the native bile duct.BDS has not been developed primarily because control of bile duct wound healing and regeneration has not been elucidated.A BDS expected to be clinically applied in the near future incorporates a bioabsorbable material that allows for regeneration of the bile duct outside the BDS.展开更多
文摘BACKGROUND Intrahepatic bile duct adenoma(BDA)is one of the rarest of the rare benign tumors of the liver in the pediatric age group.It arises from the epithelial lining of intrahepatic bile ducts.The overall incidence of BDA is 1.3%of all primary benign liver tumors.Few case reports of this rare tumor occurring in adult population are present in the literature and to date,only one pediatric case has been reported worldwide.CASE SUMMARY An 18-month-old male child presented with chief complaints of mass per abdomen for 8 mo.Computerized tomography abdomen revealed a well-defined exophytic solid tumor arising from the posteroinferior margin of the right lobe of the liver with heterogenous enhancement and cystic changes,suggesting a suspicion of hepatoblastoma.Non-anatomical liver resection was done and a tumor of 10 cm×9.5 cm was excised.Histopathology of the specimen was conclusive with the diagnosis of intrahepatic bile duct adenoma,which was further supported by immunohistochemistry panel testing.The post-operative period was uneventful.On follow-up,the child was doing well and had no postoperative complication.CONCLUSION Intrahepatic bile duct adenoma in children is very rare and to date only one case has been reported.This is the second pediatric case of intrahepatic bile duct adenoma in the world.Additionally this is the first ever case of such a large tumor presenting in a child.
文摘We report a case of a 75-year-old man with cystic micropapillary neoplasm of peribiliary glands detected preoperatively by radiologic examination. Enhanced computed tomography showed a low-density mass 2.2 cm in diameter in the right hepatic hilum and a cystic lesion around the common hepatic duct. cholangiocarcinoma, right hepatectomy with caudate lobectomy and bile duct resection were performed. Pathological examination revealed perihilar cholangiocarcinoma mainly involving the right hepatic duct. The cystic lesion was multilocular and covered by columnar lining epithelia exhibiting increased proliferative activity and p53 nuclear expression; it also contained foci of micropapillary and glandular proliferation. Therefore, the lesion was diagnosed as a cystic micropapillary neoplasm of peribiliary glands and resembled flat branch-type intraductal papillary mucinous neoplasm of the pancreas. Histological examination showed the lesion was discontinuous with the perihilar cholangiocarcinoma. Immunohistochemistry showed the cystic neoplasm was strongly positive for MUC6 and that the cholangiocarcinoma was strongly positive for MUC5 AC and S100 P. These results suggest these two lesions have different origins. This case warrants further study on whether this type of neoplasm is associated with concomitant cholangiocarcinoma as observed in pancreatic intraductal papillary mucinous neoplasm with concomitant pancreatic duct adenocarcinoma.
基金Supported by the Japan Society for the Promotion of Science,No.21K08786。
文摘Biliodigestive anastomosis between the extrahepatic bile duct and the intestine for bile duct disease is a gastrointestinal reconstruction that abolishes duodenal papilla function and frequently causes retrograde cholangitis.This chronic inflammation can cause liver dysfunction,liver abscess,and even bile duct cancer.Although research has been conducted for over 100 years to directly repair bile duct defects with alternatives,no bile duct substitute(BDS)has been developed.This narrative review confirms our understanding of why bile duct alternatives have not been developed and explains the clinical applicability of BDSs in the near future.We searched the PubMed electronic database to identify studies conducted to develop BDSs until December 2021 and identified studies in English.Two independent reviewers reviewed studies on large animals with 8 or more cases.Four types of BDSs prevail:Autologous tissue,non-bioabsorbable material,bioabsorbable material,and others(decellularized tissue,3D-printed structures,etc.).In most studies,BDSs failed due to obstruction of the lumen or stenosis of the anastomosis with the native bile duct.BDS has not been developed primarily because control of bile duct wound healing and regeneration has not been elucidated.A BDS expected to be clinically applied in the near future incorporates a bioabsorbable material that allows for regeneration of the bile duct outside the BDS.
