Humans ; Diagnosis, Differential ; Constriction, Pathologic/diagnosis* ; Bile Duct Neoplasms/diagnosis* ; Endoscopy, Digestive System

What are the new contents of the guideline since 2010?A.Patients with primary and non-primary sclerosing cholangitis (PSC) are included in these guidelines for the diagnosis and management of cholangiocarcinoma.B.Define "related stricture" as any biliary or hepatic duct stricture accompanied by the signs or symptoms of obstructive cholestasis and/or bacterial cholangitis.C.Patients who have had an inconclusive report from MRI and cholangiopancreatography should be reexamined by high-quality MRI/cholangiopancreatography for diagnostic purposes. Endoscopic retrograde cholangiopancreatography should be avoided for the diagnosis of PSC.D. Patients with PSC and unknown inflammatory bowel disease (IBD) should undergo diagnostic colonoscopic histological sampling, with follow-up examination every five years until IBD is detected.E. PSC patients with IBD should begin colon cancer monitoring at 15 years of age.F. Individual incidence rates should be interpreted with caution when using the new clinical risk tool for PSC for risk stratification.G. All patients with PSC should be considered for clinical trials; however, if ursodeoxycholic acid (13-23 mg/kg/day) is well tolerated and after 12 months of treatment, alkaline phosphatase (γ- Glutamyltransferase in children) and/or symptoms are significantly improved, it can be considered to continue to be used.H. Endoscopic retrograde cholangiopancreatography with cholangiocytology brushing and fluorescence in situ hybridization analysis should be performed on all patients suspected of having hilar or distal cholangiocarcinoma.I.Patients with PSC and recurrent cholangitis are now included in the new unified network organ sharing policy for the end-stage liver disease model standard.J. Liver transplantation is recommended after neoadjuvant therapy for patients with unresectable hilar cholangiocarcinoma with diameter < 3 cm or combined with PSC and no intrahepatic (extrahepatic) metastases.
Child ; Humans ; Cholangitis, Sclerosing/diagnosis* ; Constriction, Pathologic/complications* ; In Situ Hybridization, Fluorescence ; Cholangiocarcinoma/therapy* ; Liver Diseases/complications* ; Cholestasis ; Inflammatory Bowel Diseases/therapy* ; Bile Ducts, Intrahepatic/pathology* ; Bile Duct Neoplasms/therapy*

Objective: To investigate the utility of albumin RNAscope in situ hybridization in the diagnosis and differential diagnosis of hepatocellular carcinoma and its mimics. Methods: One hundred and fifty-two cases of hepatocellular carcinoma and its mimics and 33 cases of normal tissue were selected from the pathology database of the Department of Pathology, Ruijin Hospital, Shanghai Jiao Tong University School of Medicine from January 2013 to December 2019. Tissue microarrays were constructed and RNAscope in situ hybridization was performed to detect the expression of albumin mRNA. Results: No albumin mRNA expression was detected in normal tissues except for the liver. All hepatocellular carcinoma regardless of its degree of differentiation and primary or metastatic nature had detectable albumin mRNA, with strong and diffuse staining in 90.7% (49/54) of cases. While the positive rate of HepPar-1, Arg-1 or one of them by immunohistochemistry was 87.0% (47/54), 85.2% (46/54) and 92.6% (50/54) respectively. The positive rates of albumin mRNA in intrahepatic cholangiocarcinoma and biphenotypic hepatocellular carcinoma were 7/15 and 9/10, respectively. The former showed focal or heterogeneous staining, while the latter showed strong and diffuse staining. The positive rate of hepatoid adenocarcinoma was 8/19, and the albumin expression could be diffuse or focal. Sporadic cases of poorly differentiated gastric adenocarcinoma and metastatic colon adenocarcinoma showed focal staining of albumin mRNA. Conclusions: Detection of albumin mRNA by RNAscope in situ hybridization is of great value for the diagnosis and differential diagnosis of HCC, and the sensitivity may be improved by combining with HepPar-1 and Arg-1. It also offers different diagnostic clues according to different expression patterns.
Adenocarcinoma/diagnosis* ; Albumins/genetics* ; Bile Duct Neoplasms/pathology* ; Bile Ducts, Intrahepatic/pathology* ; Biomarkers, Tumor/genetics* ; Carcinoma, Hepatocellular/genetics* ; China ; Colonic Neoplasms ; Diagnosis, Differential ; Humans ; In Situ Hybridization ; Liver Neoplasms/pathology* ; RNA, Messenger

Objective: To establish a survival prediction model based on the independent prognostic factors of long-term prognosis after laparoscopic liver resection(LLR) for intrahepatic cholangiocarcinoma(ICC). Methods: The clinical and pathological data of 351 consecutive patients with ICC who received radical LLR in 13 Chinese medical centers from August 2010 to May 2021 were collected retrospectively. There were 190 males and 161 females,aged(M(IQR)) 61(14)years(range:23 to 93 years). The total cohort was randomly divided into a training dataset(264 cases) and a validation dataset(87 cases). The patients were followed up by outpatient service or telephone,and the deadline for follow-up was October 2021. Based on the training dataset,the multivariate Cox proportional hazards regression model was used to screen the independent influencing factors of long-term prognosis to construct a Nomogram model. The Nomogram model's discrimination,calibration,and clinical benefit were evaluated through internal and external validation,and an assessment of the overall value of two groups was made through the use of a receiver operating characteristic(ROC) curve. Results: There was no significant difference in clinical and pathological characteristics and long-term survival results between the training and validation datasets(all P>0.05). The multivariate Cox analysis showed that CA19-9,CA125,conversion to laparotomy during laparoscopic surgery,and lymph node metastasis were independent prognostic factors for ICC patients after LLR(all P<0.05). The survival Nomogram was established based on the independent prognostic factors obtained from the above screening. The ROC curve showed that the area under the curve of 1, 3 and 5-year overall survival rates of patients in the training dataset were 0.794(95%CI:0.721 to 0.867),0.728(95%CI:0.618 to 0.839) and 0.799(95%CI:0.670 to 0.928),and those in the validation dataset were 0.787(95%CI:0.660 to 0.915),0.831(95%CI:0.678 to 0.983) and 0.810(95%CI:0.639 to 0.982). Internal and external validation proved that the model exhibited a certain discrimination,calibration,and clinical applicability. Conclusion: The survival Nomogram model based on the independent influencing factors of long-term prognosis after LLR for ICC(including CA19-9,CA125,conversion to laparotomy during laparoscopic surgery,and lymph node metastasis) exhibites a certain differentiation,calibration,and clinical practicability.
Bile Duct Neoplasms/surgery* ; Bile Ducts, Intrahepatic/pathology* ; CA-19-9 Antigen ; Cholangiocarcinoma/diagnosis* ; Female ; Humans ; Laparoscopy ; Lymphatic Metastasis ; Male ; Nomograms ; Prognosis ; Retrospective Studies

The malignant degree of cholangiocarcinoma is high, and the early diagnosis is difficult. The vast majority of patients are unresectable when they are diagnosed. The patients have low quality of life and short survival cycle. Traditional radiotherapy and chemotherapy have poor efficacy and lead to side effects, and thus lack effective control measures for cholangiocarcinoma. Endoscopic retrograde cholangiopancreatography (ERCP) is an important method for diagnosing and treating biliary tract diseases. Photodynamic therapy (PDT) is a new local treatment for cholangiocarcinoma. In recent years, ERCP-mediated PDT treatment of cholangiocarcinoma has gradually emerged. ERCP-mediated PDT can effectively relieve the symptoms of patients with cholangiocarcinoma, improve the patients' quality of life, prolong the survival cycle, and is expected to become a new treatment for cholangiocarcinoma.
Bile Duct Neoplasms ; diagnosis ; Bile Ducts, Intrahepatic ; Cholangiocarcinoma ; diagnosis ; Cholangiopancreatography, Endoscopic Retrograde ; Humans ; Photochemotherapy ; Quality of Life

PURPOSE: The prognosis of periampullary cancer varies with its origin and early diagnosis influences outcome. Endoscopic ultrasound, computed tomography, fine needle aspiration, and fluorodeoxyglucose-positron emission tomography/computed tomography (¹⁸FDG-PET/CT) are helpful for diagnosis. This study evaluates the diagnostic efficacy of ¹⁸FDG-PET for preoperative periampullary tumors.METHODS: A series of 100 patients undergoing ¹⁸FDG-PET/CT before surgical resection of periampullary tumors between March 2011 and February 2019 were enrolled. Maximum standardized uptake value (SUVmax) and carbohydrate antigen 19-9 (CA19-9) levels were compared with pathohistological confirmation of periampullary tumors.RESULTS: The SUVmax and uptake range varied with the origin of the periampullary tumors. The SUVmax was not available for 17 of the 42 pancreas tumors, three of 30 common bile duct tumors, and four of 18 ampulla of Vater tumors. The median SUVmax was 0.0 in benign tumors and 5.05 in malignant tumors. The mean SUVmax was 4.1±5.6 in pancreatic tumors, 3.9±2.4 in ampulla of Vater, and 6.0±3.7 in common bile duct. The SUVmax was higher in common bile duct tumors than others. CA19-9 level was of diagnostic value in pancreatic tumor patients. The median CA19-9 levels were 7.64 U/mL (range, 2.71–45.05 U/mL) in benign tumors and 91.97 U/mL (range, 26.91–276.60 U/mL) in cancers patients.CONCLUSION: Preoperative SUVmax and CA19-9 level were of diagnostic value for periampullary tumors originating in the pancreas.
Ampulla of Vater ; Biopsy, Fine-Needle ; CA-19-9 Antigen ; Common Bile Duct ; Diagnosis ; Early Diagnosis ; Fluorodeoxyglucose F18 ; Humans ; Pancreas ; Pancreatic Neoplasms ; Prognosis ; Ultrasonography

Primary neuroendocrine tumors originating from the extrahepatic bile duct are rare. Among these tumors, large cell neuroendocrine carcinomas (NECs) are extremely rare. A 59-year-old man was admitted to Sanggye Paik Hospital with jaundice that started 10 days previously. He had a history of laparoscopic cholecystectomy, which he had undergone 12 years previously due to chronic calculous cholecystitis. Laboratory data showed abnormally elevated levels of total bilirubin 15.3 mg/dL (normal 0.2–1.2 mg/dL), AST 200 IU (normal 0–40 IU), ALT 390 IU (normal 0–40 IU), and gamma-glutamyl transferase 1,288 U/L (normal 0–60 U/L). Serum CEA was normal, but CA 19-9 was elevated 5,863 U/mL (normal 0–37 U/mL). Abdominal CT revealed a 4.5 cm sized mass involving the common bile duct and liver hilum and dilatation of both intrahepatic ducts. Percutaneous transhepatic drainage in the left hepatic duct was performed for preoperative biliary drainage. The patient underwent radical common bile duct and Roux-en-Y hepaticojejunostomy for histopathological diagnosis and surgical excision. On histopathological examination, the tumor exhibited large cell NEC (mitotic index >20/10 high-power field, Ki-67 index >20%, CD56 [+], synaptophysin [+], chromogranin [+]). Adjuvant concurrent chemotherapy and radiotherapy were started because the tumor had invaded the proximal resection margin. No recurrence was detected at 10 months by follow-up CT.
Bile Duct Neoplasms ; Bile Ducts, Extrahepatic* ; Bilirubin ; Carcinoma, Neuroendocrine* ; Cholecystectomy, Laparoscopic ; Cholecystitis ; Common Bile Duct ; Diagnosis ; Dilatation ; Drainage ; Drug Therapy ; Follow-Up Studies ; Hepatic Duct, Common ; Humans ; Jaundice ; Liver ; Middle Aged ; Neuroendocrine Tumors ; Radiotherapy ; Recurrence ; Synaptophysin ; Tomography, X-Ray Computed ; Transferases

PURPOSE: Recently, cholangioscopy using narrow band imaging (NBI) has been used as a diagnostic modality for better visualization in hepatobiliary malignancies; however, there are few reports on it. Our aim is to evaluate the effectiveness of cholangioscopy using NBI in hepatobiliary malignancies. METHODS: Between January 2007 and December 2016, 152 cholangioscopies using percutaneous approach were conducted in total 123 patients. Among these, 36 patients were suspicious of hepatobiliary malignancies. Thirteen patients with an ambiguous margin on endoscopic retrograde cholangiopancreatography (ERCP) or magnetic resonance cholangiopancreatography (MRCP), for whom NBI tipped the balance in diagnosis of lesion and decision of lesion extent by adding NBI, were involved in our study. RESULTS: Underlying diseases were all malignant in 13 patients (11 bile duct cancers, 1 liver cancer, 1 pancreas cancer with common bile duct invasion). In 7 cases with papillary type tumor, minute superficial spreading tumor was detected by NBI more easily, and NBI provided a better visualization of tumor vessel and margin evaluation in 4 cases with infiltrative tumor. In 2 cases with mucin-hypersecreting tumor, NBI showed better penetration through the mucin and gave us a much clearer image. Nine patients ultimately underwent surgical resection. The margins predicted by NBI cholangioscopy were consistent with the pathological margins on the resected specimens. CONCLUSION: In conclusion, cholangioscopy using NBI is very useful for evaluation of suspected hepatobiliary malignancies with an ambiguous margin on ERCP or MRCP. It can give us an accurate pathologic mapping, and this information seems to be essential before deciding on a treatment strategy.
Bile Duct Neoplasms ; Cholangiography ; Cholangiopancreatography, Endoscopic Retrograde ; Cholangiopancreatography, Magnetic Resonance ; Common Bile Duct ; Diagnosis ; Humans ; Liver Neoplasms ; Mucins ; Narrow Band Imaging* ; Pancreatic Neoplasms

A 54-year-old female with postprandial dyspepsia and abdominal pain was diagnosed as locally advanced unresectable intrahepatic cholangiocarcinoma by radiologic imaging studies resulting in invasion to bilateral main bile duct and right portal vein. The patient underwent extended right hepatectomy and portal vein resection after gemcitabine and cisplatin combined chemotherapy for a total of 40 cycles after the diagnosis. Final pathology showed, followed by pathological complete remission, without any residual cancer cell. The patient has survived for over 6 years without any evidence of recurrence. This case suggests that locally advanced intrahepatic cholangiocarcinoma, which can't be resected, was also proved to be capable of pathological complete remission with active chemotherapy, and long-term survival could be achieved. Therefore, active multidisciplinary approach and patient-oriented treatments using various methods should be considered for locally advanced unresectable intrahepatic cholangiocarcinoma.
Abdominal Pain ; Bile Duct Neoplasms ; Bile Ducts ; Cholangiocarcinoma* ; Cisplatin ; Diagnosis ; Drug Therapy* ; Dyspepsia ; Female ; Hepatectomy ; Humans ; Middle Aged ; Neoplasm, Residual ; Pathology ; Portal Vein ; Recurrence

BACKGROUND: Endoscopic self-expandable metal stent (SEMS) insertions for palliation of malignant biliary and duodenal obstructions have been revealed to be an effective treatment. We present our clinical experience with the use of SEMS for malignant biliary and duodenal obstructions caused by periampullary cancer. METHODS: We performed a retrospective review of all patients who underwent endoscopic biliary and duodenal SEMS insertion for palliation of malignant biliary and duodenal obstruction caused by periampullary cancer between July 2007 and October 2016. The patients received simultaneous or sequential endoscopic biliary stenting and duodenal stenting with COMVITM stents (partially covered; Taewoong, Seoul, Korea). RESULTS: The final diagnoses of our enrolled patients were 15 pancreas head cancer, and 3 distal common bile duct (CBD) cancer. The main stricture site in the duodenum was bulb (n = 4, 22.2%), 2nd portion (n = 9, 50.0%), and 3rd portion (n = 5, 27.8%). The technical success rates of CBD and duodenal stenting were 100% (18/18), and 100% (18/18), respectively. The clinical success rates of CBD and duodenal stenting were 100% (18/18), and 88.9% (16/18), respectively. Median actuarial stent patency for biliary and duodenal SEMS were 6.5 months (range, 1-2 months) and 4.5 months (range, 1-14 months), respectively. Three patients (16.7%) had recurrent biliary obstruction and all of them underwent percutaneous trans-hepatic biliary drainage (PTBD) with biliary SEMS reinsertion. Three other patients (16.7%, totally different from patients with CBD restenosis) had recurrent duodenal obstruction and all of them underwent upper gastrointestinal endoscopy with duodenal SEMS reinsertion. CONCLUSION: Endoscopic SEMS insertions for simultaneous palliation of malignant biliary and duodenal obstruction in patients with periampullary cancer may provide a safe, and less invasive alternative to surgical palliation with a successful clinical outcome.
Common Bile Duct ; Constriction, Pathologic ; Diagnosis ; Drainage ; Duodenal Obstruction ; Duodenum ; Endoscopy, Gastrointestinal ; Head and Neck Neoplasms ; Humans ; Palliative Care* ; Pancreas ; Retrospective Studies ; Self Expandable Metallic Stents ; Seoul ; Stents*