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*

Aged ; Bile Duct Diseases ; complications ; diagnostic imaging ; pathology ; Bile Duct Neoplasms ; diagnosis ; Carcinoma, Hepatocellular ; complications ; diagnostic imaging ; pathology ; Diagnosis, Differential ; Humans ; Jaundice, Obstructive ; etiology ; Klatskin Tumor ; diagnosis ; Liver Neoplasms ; complications ; diagnostic imaging ; pathology ; Male ; Middle Aged ; Thrombosis ; complications ; diagnostic imaging ; pathology ; Tomography, X-Ray Computed

A 51-year-old male patient with chronic hepatitis B was referred to our hospital due to a 1-cm liver nodule on ultrasonography. Alpha-fetoprotein (AFP) was slightly elevated. The nodule showed prolonged enhancement on dynamic liver magnetic resonance imaging and appeared as a hyperintensity on both diffusion-weighted and T2-weighted imaging. The nodule was followed up because it was small and typical findings of hepatocellular carcinoma (HCC) were not observed in the dynamic imaging investigations. However, liver contrast-enhanced ultrasonography performed 1 month later showed enhancement during the arterial phase and definite washout during the delayed phase. Also, AFP had increased to over 200 ng/mL even though AST and ALT were decreased after administering an antiviral agent. He was presumptively diagnosed as HCC and underwent liver segmentectomy. Microscopy findings of the specimen indicated bile duct adenoma. After resection, the follow-up AFP had decreased to within the normal range. This patient represents a case of bile duct adenoma with AFP elevation mimicking HCC on contrast-enhanced ultrasonography.
Bile Duct Neoplasms/*complications/*diagnosis/pathology ; *Bile Ducts, Intrahepatic ; Hepatitis B, Chronic/*complications/*diagnosis/pathology ; Humans ; Liver/pathology/ultrasonography ; Magnetic Resonance Imaging ; Male ; Middle Aged ; Tomography, X-Ray Computed ; alpha-Fetoproteins/*metabolism

No abstract available.
Bile Duct Neoplasms/complications/*diagnosis ; *Bile Ducts, Intrahepatic/chemistry/pathology/radiography ; Biopsy ; Cholangiocarcinoma/complications/*diagnosis ; Diagnosis, Differential ; Fever/diagnosis/*etiology ; Humans ; Immunohistochemistry ; Leukocytosis/*diagnosis/etiology ; *Liver/chemistry/pathology/radiography ; Liver Abscess, Pyogenic/*diagnosis ; Magnetic Resonance Imaging ; Male ; Middle Aged ; Paraneoplastic Syndromes/*diagnosis/etiology ; Predictive Value of Tests ; Tomography, Spiral Computed ; Tumor Markers, Biological/analysis

BACKGROUND/AIMS: There are several methods for obtaining tissue samples to diagnose malignant biliary strictures during endoscopic retrograde cholangiopancreatography (ERCP). However, each method has only limited sensitivity. This study aimed to evaluate the diagnostic accuracy of a combined triple-tissue sampling (TTS) method (on-site bile aspiration cytology, brush cytology, and forceps biopsy). METHODS: We retrospectively reviewed 168 patients with suspicious malignant biliary strictures who underwent double-tissue sampling (DTS; n=121) or TTS (n=47) via ERCP at our institution from 2004 to 2011. RESULTS: Among the 168 patients reviewed, 117 patients (69.6%) were eventually diagnosed with malignancies. The diagnostic sensitivity for cancer was significantly higher in the TTS group than the DTS group (85.0% vs 64.9%, respectively; p=0.022). Furthermore, the combination of brush cytology and forceps biopsy was superior to the other method combinations in the DTS group. With respect to cancer type (cholangiocarcinoma vs noncholangiocarcinoma), interestingly, the diagnostic sensitivity was higher for cholangiocarcinoma in the TTS group than the DTS group (100% vs 69.4%, respectively; p<0.001) but not for the non-cholangiocarcinoma patients (57.1% vs 57.1%, respectively). CONCLUSIONS: TTS can provide an improved diagnostic accuracy in suspicious malignant biliary strictures, particularly for cholangiocarcinoma.
Aged ; Ampulla of Vater/*pathology ; Bile Duct Neoplasms/complications/diagnosis/pathology ; Bile Ducts, Intrahepatic/*pathology ; Biopsy/*methods ; Biopsy, Needle ; Carcinoma/complications/diagnosis/pathology ; Cholangiocarcinoma/complications/*diagnosis/pathology ; Cholangiopancreatography, Endoscopic Retrograde/*methods ; Cholestasis/etiology ; Common Bile Duct Neoplasms/complications/*diagnosis/pathology ; Female ; Humans ; Male ; Middle Aged ; Pancreatic Neoplasms/complications/*diagnosis/pathology ; Retrospective Studies ; Sensitivity and Specificity

Extrahepatic cholangiocarcinoma is often clinically challenging to diagnose. Even multidisciplinary approaches which include computed tomography, magnetic resonance imaging, and endoscopic retrograde cholangiography are unsatisfactory in some cases, especially with biliary stricture. Percutaneous transhepatic cholangioscopy (PTCS) with its direct visualization for biopsy appears to be a promising technique for detecting cholangiocarcinoma at an early stage. We report a case of adenocarcinoma in situ of the distal common bile duct (CBD) that was confirmed by PTCS. This case suggests the useful role of PTCS in the differential diagnosis of a distal CBD obstruction, particularly when other diagnostic modalities do not provide definitive information.
Carcinoma in Situ/complications/*diagnosis/pathology/surgery ; Cholangiocarcinoma/complications/*diagnosis/pathology/surgery ; Cholangiopancreatography, Endoscopic Retrograde ; Cholestasis, Extrahepatic/diagnosis/etiology ; Common Bile Duct/*pathology/surgery ; Common Bile Duct Neoplasms/complications/*diagnosis/pathology/surgery ; Constriction, Pathologic ; *Endoscopy, Digestive System ; Humans ; Male ; Middle Aged ; Pancreaticoduodenectomy ; Predictive Value of Tests ; Tomography, X-Ray Computed ; Treatment Outcome

Anomalous union of the pancreaticobiliary duct (AUPBD) is a congenital anomaly that is defined as a junction of the bile duct and pancreatic duct outside the duodenal wall. This anomaly results in a loss of normal sphincteric mechanisms at the pancreaticobiliary junction. As a result, regurgitation of pancreatic juice into the biliary system develops and causes choledochal cysts, choledocholithiasis, cholangitis, pancreatitis and malignancy of the biliary tract. Gallbladder cancer or common bile duct cancer associated with AUPBD and choledochal cysts have been frequently reported. But, intrahepatic cholangiocarcinoma associated with this condition has been only rarely reported. Here, we report a case of intrahepatic cholangiocarcinoma associated with AUPBD and choledochal cyst.
Adult ; Bile Duct Neoplasms/*diagnosis/etiology/pathology ; *Bile Ducts, Intrahepatic ; Cholangiocarcinoma/*diagnosis/etiology/pathology ; Cholangiopancreatography, Endoscopic Retrograde ; Cholangiopancreatography, Magnetic Resonance ; Choledochal Cyst/complications/*diagnosis ; Female ; Humans ; Positron-Emission Tomography and Computed Tomography ; Tomography, X-Ray Computed

An 80-year-old woman with hilar cholangiocarcinoma was hospitalized due to sudden-onset abdominal pain. Computed tomography revealed hepatic necrosis accompanied with emphysematous change in the superior segment of the right liver (S7/S8), implying spontaneous rupture, based on the presence of perihepatic free air. Although urgent percutaneous drainage was performed, neither pus nor fluids were drained. These findings suggest emphysematous hepatitis with a hepatic mass. Despite the application of intensive care, the patient's condition deteriorated rapidly, and she died 3 days after admission to hospital. Liver gas has been reported in some clinical diseases (e.g., liver abscess) to be caused by gas-forming organisms; however, emphysematous hepatitis simulating emphysematous pyelonephritis is very rare. The case reported here was of fatal emphysematous hepatitis in a patient with hilar cholangiocarcinoma.
Aged, 80 and over ; Anti-Bacterial Agents/therapeutic use ; Bile Duct Neoplasms/complications/diagnosis ; Bile Ducts, Intrahepatic/pathology ; Cefotaxime/therapeutic use ; *Cholangiocarcinoma/complications/diagnosis ; Clostridium Infections/drug therapy/microbiology ; Clostridium perfringens/isolation & purification ; Emphysema/complications/*diagnosis ; Escherichia coli/isolation & purification ; Escherichia coli Infections/drug therapy/microbiology ; Female ; Hepatitis/complications/*diagnosis ; Humans ; Metronidazole/therapeutic use ; *Pneumoperitoneum/complications/diagnosis ; Tomography, X-Ray Computed

Henoch-Schonlein purpura (HSP) is common in childhood and often self-limiting. There have been limited studies on elderly-onset HSP nephritis (HSPN). A 76-yr-old man was transferred to our hospital with a 1-month history of oliguria, abdominal pain, edema and palpable purpura in the legs. Three months ago, he was admitted to another hospital with jaundice, and consequently diagnosed with early common bile duct cancer. The patient underwent a Whipple's operation. Antibiotics were administrated because of leakage in the suture from the surgery. However, he showed progressive renal failure with edema and purpura in the legs. Laboratory investigations showed serum creatinine 6.4 mg/dL, 24-hr urine protein 8,141 mg/day, myeloperoxidase anti-neutrophil cytoplasmic antibodies (MPO-ANCA) 1:40 and C3 below 64.89 mg/dL. Renal biopsy showed crescentic glomerulonephritis, as well as mesangial and extracapillary Ig A deposition. We started steroid therapy and hemodialysis, but he progressed to end-stage renal failure and he has been under maintenance hemodialysis. We describe elderly onset HSPN with MPO-ANCA can be crescentic glomerulonephritis rapidly progressed to end stage renal failure.
Aged ; Antibodies, Antineutrophil Cytoplasmic/*analysis ; Common Bile Duct Neoplasms/complications/surgery ; Complement C3/analysis ; Creatinine/blood ; Edema/drug therapy ; Enzyme-Linked Immunosorbent Assay ; Glomerulonephritis/pathology ; Humans ; Male ; Purpura, Schoenlein-Henoch/*diagnosis/drug therapy ; Renal Dialysis ; Renal Insufficiency/etiology/pathology ; Steroids/therapeutic use

We report a case of small cell carcinoma of extrahepatic bile duct presenting with jaundice and hemobilia. A 59-year-old woman was admitted due to right upper quadrant pain and jaundice. An abdominal computed tomography revealed a 2 cm sized mass in the extrahepatic bile duct. Endoscopic retrograde cholangiopancreatography revealed bloody discharge coming out of the papillary orifice in endoscopic view and a dilated extrahepatic bile duct with multiple irregular filling defects in cholangiogram. A coronal T2-weighted image revealed a hyperintense mass at extrahepatic bile duct. Laparotomy was performed, and pathologic examination of resected specimen showed tumor cells having round to oval nuclei with coarsely granular chromatin and scanty cytoplasm, which were immunoreactive for synaptophysin and chromogranin A, compatible with the diagnosis of small cell carcinoma. The small cell carcinoma of bile duct, despite its rarity, should be considered in differential diagnosis of the causes for obstructive jaundice and hemobilia.
Bile Duct Neoplasms/*diagnosis/pathology/radiography ; Bile Ducts, Extrahepatic/*pathology/radiography ; Carcinoma, Small Cell/*diagnosis/pathology/radiography ; Cholangiopancreatography, Endoscopic Retrograde ; Chromogranin A/metabolism ; Female ; Hemobilia/complications/*diagnosis ; Humans ; Magnetic Resonance Imaging ; Middle Aged ; Synaptophysin/metabolism ; Tomography, X-Ray Computed