The natural history,associations with inflammatory bowel disease(IBD),and long-term outcomes of large duct primary sclerosing cholangitis(ldPSC)have been well documented.Small duct primary sclerosing cholangitis(sdPSC...The natural history,associations with inflammatory bowel disease(IBD),and long-term outcomes of large duct primary sclerosing cholangitis(ldPSC)have been well documented.Small duct primary sclerosing cholangitis(sdPSC)is a much less common and relatively more benign variant.The natural history of sdPSC has been difficult to characterize given the limited number of studies in the literature especially with regards to the subset of patients who progress to large duct involvement.It has been unclear whether sdPSC represented a subset of ldPSC,an earlier staging of ldPSC,or a completely separate and distinct entity of its own.Strong associations between sdPSC and IBD have been established with suspicion that concurrent sdPSC-IBD may be a key prognostic factor in determining which patients are at risk of progression to ldPSC.Little is known regarding the discrete circumstances that predisposes some patients with sdPSC to progress to ldPSC.It has been suspected that progression to large biliary duct involvement subjects this subset of patients to potentially developing lifethreatening complications.Here the authors conducted a thorough review of the published sdPSC literature using Pubmed searches and cross-referencing to compile all accessible studies regarding cohorts of sdPSC patients in order better characterize the subset of sdPSC patients who progress to ldPSC and the associated outcomes.展开更多
Common bile duct stones are among the most common conditions encountered by endoscopists.Therefore,it is well researched;however,some items,such as indications for endoscopic papillary balloon dilatation(EPBD),safety ...Common bile duct stones are among the most common conditions encountered by endoscopists.Therefore,it is well researched;however,some items,such as indications for endoscopic papillary balloon dilatation(EPBD),safety of EPBD and endoscopic sphincterotomy in patients receiving dual antiplatelet therapy or direct oral anticoagulant,selection strategy for retrieval balloons and baskets,lack adequate evidence.Therefore,the guidelines have been updated with new research,while others remain unchanged due to weak evidence.In this review,we comprehensively summarize the standard methods in guidelines and new findings from recent studies on papillary dilation,stone retrieval devices,difficult-to-treat cases,troubleshooting during the procedure,and complicated cases of cholangitis,cholecystolithiasis,or distal biliary stricture.展开更多
AIM:To investigate the clinicopathologic features of bile duct tumor thrombus(BDTT) occurrence after treatment of primary small hepatocellular carcinoma(sHCC) .METHODS:A total of 423 patients with primary sHCC admitte...AIM:To investigate the clinicopathologic features of bile duct tumor thrombus(BDTT) occurrence after treatment of primary small hepatocellular carcinoma(sHCC) .METHODS:A total of 423 patients with primary sHCC admitted to our hospital underwent surgical resection or local ablation.During follow-up,only six patients were hospitalized due to obstructive jaundice,which occurred 5-76 mo after initial treatment.The clinicopathologic features of these six patients were reviewed.RESULTS:Six patients underwent hepatic resection(n=5) or radio-frequency ablation(n=1) due to primary sHCC.Five cases had an R1 resection margin,and one case had an ablative margin less than 5.0 mm.No vascular infiltration,microsatellites or bile duct/canaliculus affection was noted in the initial resected specimens.During the follow-up,imaging studies revealed a macroscopic BDTT extending to the common bile duct in all six patients.Four patients had a concomitant intrahepatic recurrent tumor.Surgical re-resection of intrahepatic recurrent tumors and removal of BDTTs(n=4) ,BDTT removal through choledochotomy(n= 1) ,and conservative treatment(n=1) was performed.Microscopic portal vein invasion was noted in three of the four resected specimens.All six patients died,with a mean survival of 11 mo after BDTT removal or conservative treatment.CONCLUSION:BDTT occurrence is a rare,special recurrent pattern of primary sHCC.Patients with BDTTs extending to the common bile duct usually have an unfavorable prognosis even following aggressive surgery.Insufficient resection or ablative margins against primary sHCC may be a risk factor for BDTT development.展开更多
BACKGROUND:Primary sclerosing cholangitis(PSC)is a chronic progressive cholestatic liver disease,which usually affects young adults and is diagnosed by cholangiography.On a few occasions,the disease either starts in o...BACKGROUND:Primary sclerosing cholangitis(PSC)is a chronic progressive cholestatic liver disease,which usually affects young adults and is diagnosed by cholangiography.On a few occasions,the disease either starts in or exclusively involves the small intrahepatic bile ducts,referred to as small-duct PSC. METHODS:A 31-year-old man presented with severe hematemesis secondary to liver cirrhosis.Over a course of 8 years,his liver decompensated and required an orthotopic liver transplantation. In this report we discuss his disease presentation,course of management,and the post-transplantation course of manage- ment,and review the morphologic diagnosis,and differential diagnosis of the disease with large-duct type and other diseases that involve small intrahepatic bile ducts. RESULTS:The patient’s explanted liver showed changes of PSC affecting only the small-and medium-sized bile ducts in addition to three incidental nodules of hepatocellular carcinoma. CONCLUSIONS:Small-duct PSC has a substantially better prognosis than the large-duct type,with less chance of developing cirrhosis and an equal risk for developing hepato- cellular carcinoma,but no increased risk for developing cholangiocarcinoma.Treatment seems to help relieve the symptoms but not necessarily improve survival.Liver transplantation remains the ultimate cure.展开更多
Neuroendocrine carcinoma (NEC) of the extrahepatic bile duct is rare, and only 22 cases have been reported. Only two of these were large-cell NEC (LCNEC); the vast majority were small-cell NEC. Here, we report a third...Neuroendocrine carcinoma (NEC) of the extrahepatic bile duct is rare, and only 22 cases have been reported. Only two of these were large-cell NEC (LCNEC); the vast majority were small-cell NEC. Here, we report a third case of LCNEC of the extrahepatic bile duct. A 76-year-old male presented to a local hospital with painless jaundice. Imaging studies revealed a tumor at the hepatic hilum. The patient underwent right hepatic lobectomy, bile duct resection, and cholecystectomy. The resection specimen showed a 5.0-cm invasive neoplasm involving the hilar bile ducts and surrounding soft tissue. Histologically, the tumor consisted of nests of medium to large cells with little intervening stroma. The tumor invaded a large portal vein branch. All four excised lymph nodes were positive for metastasis, and metastatic deposits were also present in the gallbladder wall. The tumor was diffusely positive for synaptophysin and focally positive for chromogranin A. Approximately 70%-80% of the tumor cells were positive for Ki-67, indicating strong proliferative activity. A diagnosis of LCNEC was made. A few bile ducts within and adjacent to the invasive tumor showed dysplasia of the intestinal phenotype and were focally positive for synaptophysin and chromogranin A, suggesting that the dysplastic intestinal-type epithelium played a precursor role in this case. A postoperative computer tomography scan revealed rapid enlargement of the abdominal and retroperitoneal lymph nodes. The patient died 21 d after the operation. NEC of the bile duct is an aggressive neoplasm, and its biological characteristics remain to be better defined.展开更多
An 80-year-old woman presenting with chest pain was found to have a large,lobulated soft tissue mass in the liver and nearby tissues on abdominal computed tomography(CT).The tumor had invaded the common hepatic artery...An 80-year-old woman presenting with chest pain was found to have a large,lobulated soft tissue mass in the liver and nearby tissues on abdominal computed tomography(CT).The tumor had invaded the common hepatic artery and main portal vein.Jaundice developed 4 wk later,at which point,a pancreas and biliary CT scan revealed a large mass in the right lobe of the liver and a hilar duct obstruction,which was found to be a small cell carcinoma.Despite its rarity,liver and bile duct small cell carcinoma should be considered in the differential diagnosis of atypical chest pain without jaundice.展开更多
This paper investigates the reasoning, based on both Chinese and Western medical data, which will lead to an understanding of the relation of the heart and small intestine, organs which Chinese Medicine, in the Fire e...This paper investigates the reasoning, based on both Chinese and Western medical data, which will lead to an understanding of the relation of the heart and small intestine, organs which Chinese Medicine, in the Fire energy phase, link both functionally and anatomically. The direct relationship between the liver and the gall bladder and between the kidneys and the bladder is recognised and accepted in both Chinese and Western Medicine. This is not the case with the pairings which in Eastern morphophisiology are formed by the heart and small intestine and the lungs and large intestine. These pairings are not recognised in Western Medicine. The writer in her dual capacity of Doctor of Western Medicine and acupuncturist is investigating the reasons why in Chinese Medicine the heart and small intestine and their meridians form a relation which couples them. For this the comparative method was used between data from Western anatomy which demonstrate the interorganic and functional relation between the small intestine and the heart and the Chinese energy dynamic of the corresponding zangfu and jingluo. Biomedicine which does not relate the heart with the small intestine brings in the materiality of its anatomic descriptions which are valuable for the interpretation of Oriental Medicine. This interrelation between the two organs and their meridians are well explicated in Chinese Medicine whose traditional concepts in this respect are corroborated by Western anatomical descriptions which, nevertheless, do not admit the functional-organic coupling of the heart and small intestine.展开更多
Thyroid transcription factor 1 (TTF-1) plays a key role in morphogenesis of the lungs and is expressed in up to 90% of pulmonary small cell carcinomas.This explains why this marker is frequently used in the search for...Thyroid transcription factor 1 (TTF-1) plays a key role in morphogenesis of the lungs and is expressed in up to 90% of pulmonary small cell carcinomas.This explains why this marker is frequently used in the search for the primary origin of metastatic endocrine tumours.Here we report on a TTF-1 expressing mixed endocrine-exocrine carcinoma of the common bile duct in a patient with pulmonary nodules that did not appear to be neoplastic.TTF-1 positivity in pulmonary and extrapulmonary neuroendocrine tumours is reviewed,and we conclude that TTF-1 expression in neuroendocrine tumours of the small-cell type are not uncommon at extrapulmonary locations.Therefore,immunohistochem-istry for TTF-1 in such tumours should be interpreted with caution.展开更多
BACKGROUND The combination of immune checkpoint inhibitors and chemotherapy has shown promising results for the treatment of advanced biliary tract cancer(BTC).Based on the results of the TOPAZ-1 trial,a gemcitabine a...BACKGROUND The combination of immune checkpoint inhibitors and chemotherapy has shown promising results for the treatment of advanced biliary tract cancer(BTC).Based on the results of the TOPAZ-1 trial,a gemcitabine and cisplatin plus durvalumab(GCD)regimen was recently approved as first-line therapy for patients with advanced BTC.However,post-GCD conversion surgery has not been previously studied.Herein,we describe a case of advanced intrahepatic cholangiocarcinoma(ICC)successfully treated with radical surgery after GCD.CASE SUMMARY A 65-year-old female diagnosed with advanced ICC with periductal infiltration into the hepatic hilum underwent eight cycles of GCD,followed by durvalumab maintenance treatment,with mild adverse events.Partial response was obtained.Subsequently,a conversion surgery with extended left hepatectomy and bile duct resection was performed.The resection margins were negative,and the pathological diagnosis was compatible with small duct type ICC.The patient remained disease-free for 8 months without adjuvant chemotherapy.CONCLUSION We describe the case of a patient who received successful conversion surgery after GCD treatment for advanced ICC.展开更多
文摘The natural history,associations with inflammatory bowel disease(IBD),and long-term outcomes of large duct primary sclerosing cholangitis(ldPSC)have been well documented.Small duct primary sclerosing cholangitis(sdPSC)is a much less common and relatively more benign variant.The natural history of sdPSC has been difficult to characterize given the limited number of studies in the literature especially with regards to the subset of patients who progress to large duct involvement.It has been unclear whether sdPSC represented a subset of ldPSC,an earlier staging of ldPSC,or a completely separate and distinct entity of its own.Strong associations between sdPSC and IBD have been established with suspicion that concurrent sdPSC-IBD may be a key prognostic factor in determining which patients are at risk of progression to ldPSC.Little is known regarding the discrete circumstances that predisposes some patients with sdPSC to progress to ldPSC.It has been suspected that progression to large biliary duct involvement subjects this subset of patients to potentially developing lifethreatening complications.Here the authors conducted a thorough review of the published sdPSC literature using Pubmed searches and cross-referencing to compile all accessible studies regarding cohorts of sdPSC patients in order better characterize the subset of sdPSC patients who progress to ldPSC and the associated outcomes.
文摘Common bile duct stones are among the most common conditions encountered by endoscopists.Therefore,it is well researched;however,some items,such as indications for endoscopic papillary balloon dilatation(EPBD),safety of EPBD and endoscopic sphincterotomy in patients receiving dual antiplatelet therapy or direct oral anticoagulant,selection strategy for retrieval balloons and baskets,lack adequate evidence.Therefore,the guidelines have been updated with new research,while others remain unchanged due to weak evidence.In this review,we comprehensively summarize the standard methods in guidelines and new findings from recent studies on papillary dilation,stone retrieval devices,difficult-to-treat cases,troubleshooting during the procedure,and complicated cases of cholangitis,cholecystolithiasis,or distal biliary stricture.
文摘AIM:To investigate the clinicopathologic features of bile duct tumor thrombus(BDTT) occurrence after treatment of primary small hepatocellular carcinoma(sHCC) .METHODS:A total of 423 patients with primary sHCC admitted to our hospital underwent surgical resection or local ablation.During follow-up,only six patients were hospitalized due to obstructive jaundice,which occurred 5-76 mo after initial treatment.The clinicopathologic features of these six patients were reviewed.RESULTS:Six patients underwent hepatic resection(n=5) or radio-frequency ablation(n=1) due to primary sHCC.Five cases had an R1 resection margin,and one case had an ablative margin less than 5.0 mm.No vascular infiltration,microsatellites or bile duct/canaliculus affection was noted in the initial resected specimens.During the follow-up,imaging studies revealed a macroscopic BDTT extending to the common bile duct in all six patients.Four patients had a concomitant intrahepatic recurrent tumor.Surgical re-resection of intrahepatic recurrent tumors and removal of BDTTs(n=4) ,BDTT removal through choledochotomy(n= 1) ,and conservative treatment(n=1) was performed.Microscopic portal vein invasion was noted in three of the four resected specimens.All six patients died,with a mean survival of 11 mo after BDTT removal or conservative treatment.CONCLUSION:BDTT occurrence is a rare,special recurrent pattern of primary sHCC.Patients with BDTTs extending to the common bile duct usually have an unfavorable prognosis even following aggressive surgery.Insufficient resection or ablative margins against primary sHCC may be a risk factor for BDTT development.
文摘BACKGROUND:Primary sclerosing cholangitis(PSC)is a chronic progressive cholestatic liver disease,which usually affects young adults and is diagnosed by cholangiography.On a few occasions,the disease either starts in or exclusively involves the small intrahepatic bile ducts,referred to as small-duct PSC. METHODS:A 31-year-old man presented with severe hematemesis secondary to liver cirrhosis.Over a course of 8 years,his liver decompensated and required an orthotopic liver transplantation. In this report we discuss his disease presentation,course of management,and the post-transplantation course of manage- ment,and review the morphologic diagnosis,and differential diagnosis of the disease with large-duct type and other diseases that involve small intrahepatic bile ducts. RESULTS:The patient’s explanted liver showed changes of PSC affecting only the small-and medium-sized bile ducts in addition to three incidental nodules of hepatocellular carcinoma. CONCLUSIONS:Small-duct PSC has a substantially better prognosis than the large-duct type,with less chance of developing cirrhosis and an equal risk for developing hepato- cellular carcinoma,but no increased risk for developing cholangiocarcinoma.Treatment seems to help relieve the symptoms but not necessarily improve survival.Liver transplantation remains the ultimate cure.
文摘Neuroendocrine carcinoma (NEC) of the extrahepatic bile duct is rare, and only 22 cases have been reported. Only two of these were large-cell NEC (LCNEC); the vast majority were small-cell NEC. Here, we report a third case of LCNEC of the extrahepatic bile duct. A 76-year-old male presented to a local hospital with painless jaundice. Imaging studies revealed a tumor at the hepatic hilum. The patient underwent right hepatic lobectomy, bile duct resection, and cholecystectomy. The resection specimen showed a 5.0-cm invasive neoplasm involving the hilar bile ducts and surrounding soft tissue. Histologically, the tumor consisted of nests of medium to large cells with little intervening stroma. The tumor invaded a large portal vein branch. All four excised lymph nodes were positive for metastasis, and metastatic deposits were also present in the gallbladder wall. The tumor was diffusely positive for synaptophysin and focally positive for chromogranin A. Approximately 70%-80% of the tumor cells were positive for Ki-67, indicating strong proliferative activity. A diagnosis of LCNEC was made. A few bile ducts within and adjacent to the invasive tumor showed dysplasia of the intestinal phenotype and were focally positive for synaptophysin and chromogranin A, suggesting that the dysplastic intestinal-type epithelium played a precursor role in this case. A postoperative computer tomography scan revealed rapid enlargement of the abdominal and retroperitoneal lymph nodes. The patient died 21 d after the operation. NEC of the bile duct is an aggressive neoplasm, and its biological characteristics remain to be better defined.
文摘An 80-year-old woman presenting with chest pain was found to have a large,lobulated soft tissue mass in the liver and nearby tissues on abdominal computed tomography(CT).The tumor had invaded the common hepatic artery and main portal vein.Jaundice developed 4 wk later,at which point,a pancreas and biliary CT scan revealed a large mass in the right lobe of the liver and a hilar duct obstruction,which was found to be a small cell carcinoma.Despite its rarity,liver and bile duct small cell carcinoma should be considered in the differential diagnosis of atypical chest pain without jaundice.
文摘This paper investigates the reasoning, based on both Chinese and Western medical data, which will lead to an understanding of the relation of the heart and small intestine, organs which Chinese Medicine, in the Fire energy phase, link both functionally and anatomically. The direct relationship between the liver and the gall bladder and between the kidneys and the bladder is recognised and accepted in both Chinese and Western Medicine. This is not the case with the pairings which in Eastern morphophisiology are formed by the heart and small intestine and the lungs and large intestine. These pairings are not recognised in Western Medicine. The writer in her dual capacity of Doctor of Western Medicine and acupuncturist is investigating the reasons why in Chinese Medicine the heart and small intestine and their meridians form a relation which couples them. For this the comparative method was used between data from Western anatomy which demonstrate the interorganic and functional relation between the small intestine and the heart and the Chinese energy dynamic of the corresponding zangfu and jingluo. Biomedicine which does not relate the heart with the small intestine brings in the materiality of its anatomic descriptions which are valuable for the interpretation of Oriental Medicine. This interrelation between the two organs and their meridians are well explicated in Chinese Medicine whose traditional concepts in this respect are corroborated by Western anatomical descriptions which, nevertheless, do not admit the functional-organic coupling of the heart and small intestine.
基金Supported by The"Fondation Yvonne Bo l"(Brussels,Bel-gium)
文摘Thyroid transcription factor 1 (TTF-1) plays a key role in morphogenesis of the lungs and is expressed in up to 90% of pulmonary small cell carcinomas.This explains why this marker is frequently used in the search for the primary origin of metastatic endocrine tumours.Here we report on a TTF-1 expressing mixed endocrine-exocrine carcinoma of the common bile duct in a patient with pulmonary nodules that did not appear to be neoplastic.TTF-1 positivity in pulmonary and extrapulmonary neuroendocrine tumours is reviewed,and we conclude that TTF-1 expression in neuroendocrine tumours of the small-cell type are not uncommon at extrapulmonary locations.Therefore,immunohistochem-istry for TTF-1 in such tumours should be interpreted with caution.
文摘BACKGROUND The combination of immune checkpoint inhibitors and chemotherapy has shown promising results for the treatment of advanced biliary tract cancer(BTC).Based on the results of the TOPAZ-1 trial,a gemcitabine and cisplatin plus durvalumab(GCD)regimen was recently approved as first-line therapy for patients with advanced BTC.However,post-GCD conversion surgery has not been previously studied.Herein,we describe a case of advanced intrahepatic cholangiocarcinoma(ICC)successfully treated with radical surgery after GCD.CASE SUMMARY A 65-year-old female diagnosed with advanced ICC with periductal infiltration into the hepatic hilum underwent eight cycles of GCD,followed by durvalumab maintenance treatment,with mild adverse events.Partial response was obtained.Subsequently,a conversion surgery with extended left hepatectomy and bile duct resection was performed.The resection margins were negative,and the pathological diagnosis was compatible with small duct type ICC.The patient remained disease-free for 8 months without adjuvant chemotherapy.CONCLUSION We describe the case of a patient who received successful conversion surgery after GCD treatment for advanced ICC.
