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*

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

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

Biliary papillomatosis is rare, and its pathogenic mechanisms are not yet clear. Because of its high risk for malignancy transformation, surgical resection is regarded as a standard treatment. Photodynamic therapy (PDT) has been used by the intravenous administration of hematoporphyrin derivative followed by laser exposure. A photochemical process causes disturbance of the microvascular structure and degradation of membrane. Cholangitis is a major complication after PDT. A healthy 56-year-old man was diagnosed with biliary papillomatosis involving the common hepatic duct, both proximal intrahepatic bile ducts (IHD), and the right posterior IHD. After biliary decompression by endoscopic nasobiliary drainage, PDT was performed to avoid extensive liver resection and recurrence using endoscopic retrograde cholangiographic guidance. After portal vein embolization, the patient underwent extended right hemihepatectomy. Following administration of chemoradiation therapy with tegafur-uracil and 45 Gy due to local recurrence at postoperative 13 months, there was no local recurrence or distant metastases. This is the first case report on PDT for biliary papillomatosis in Korea. Preoperative PDT is beneficial for reducing the lesion in diffuse or multifocal biliary papillomatosis and may lead to curative and volume reserving surgery. Thus, PDT could improve the quality of life and prolong life expectation for biliary papillomatosis patients.
Antineoplastic Agents/therapeutic use ; Bile Duct Neoplasms/*diagnosis/drug therapy/surgery ; Bile Ducts, Intrahepatic/pathology ; Embolization, Therapeutic ; Gamma Rays ; Hepatectomy ; Hepatic Duct, Common/pathology ; Humans ; Male ; Middle Aged ; Neoplasm Recurrence, Local ; Papilloma/*diagnosis/drug therapy/surgery ; Photochemotherapy ; Tegafur/therapeutic use ; Uracil/therapeutic use

The patient was a 70-year-old male whose chief complaints were obstructive jaundice and weight loss. Abdominal imaging studies showed a 2.5 cm sized mass at the distal common bile duct, which was suggestive of bile duct cancer. Eccentric enhancing wall thickening in the transverse colon was also shown, suggesting concomitant colon cancer. A colonoscopy revealed a lumen-encircling ulcerofungating mass in the transverse colon, that was pathologically proven to be adenocarcinoma. The bile duct pathology was also adenocarcinoma. Pylorus-preserving pancreaticoduodenectomy and extended right hemicolectomy were performed under the diagnosis of double primary cancers. Postoperative histopathologic examination revealed moderately differentiated mucinous adenocarcinoma of transverse colon cancer, and mucinous adenocarcinoma of the distal common bile duct. Immunohistochemical staining studies showed that the bile duct cancer had metastasized from the colon cancer. The patient recovered uneventfully from surgery and will be undergoing chemotherapy for three months.
Adenocarcinoma ; Adenocarcinoma, Mucinous* ; Aged ; Bile Duct Neoplasms ; Bile Ducts ; Colon, Transverse* ; Colonic Neoplasms ; Colonoscopy ; Common Bile Duct Neoplasms ; Common Bile Duct* ; Diagnosis ; Drug Therapy ; Humans ; Jaundice, Obstructive* ; Male ; Mucins* ; Neoplasm Metastasis ; Pancreaticoduodenectomy ; Pathology ; Weight Loss

Invasion of the bile duct by hepatocellular carcinoma (HCC), which is called intrahepatic bile duct HCC, is rare and has a poor prognosis. Early diagnosis and surgical resection is important for treatment. A 58-year-old man who underwent hepatic resection for HCC 4 years ago and received transarterial chemoembolization (TACE) 2 years after the operation for recurred HCC presented with jaundice. CT scan revealed a tumor in the common bile duct without intrahepatic lesion. Therefore, ERCP was done to perform biopsy and biliary drainage. Histological examination was compatible with hepatocellular carcinoma. However, the tumor could not be visualized at angiography and thus, only transarterial chemoinfusion was performed without embolization. The tumor had disappeared on follow-up CT scan, and the patient has been disease free for 23 months without evidence of recurrence. Herein, we report a case of intrahepatic bile duct HCC which disappeared after ERCP.
Antibiotics, Antineoplastic/therapeutic use ; Bile Duct Neoplasms/diagnosis/pathology/secondary/*therapy ; Bile Ducts, Intrahepatic ; Carcinoma, Hepatocellular/*diagnosis/pathology ; Cholangiopancreatography, Endoscopic Retrograde ; Doxorubicin/therapeutic use ; Embolization, Therapeutic ; Ethiodized Oil/therapeutic use ; Humans ; Jaundice/etiology ; Liver Neoplasms/*diagnosis/pathology ; Male ; Middle Aged ; Neoplasm Recurrence, Local ; Stents ; Tomography, X-Ray Computed ; Treatment Outcome

No abstract available.
Bile Duct Neoplasms/*diagnosis/pathology/radiography ; Carcinoma, Hepatocellular/*diagnosis/radiography/therapy ; Chemoembolization, Therapeutic ; Cholangiocarcinoma/*diagnosis/pathology/radiography ; Humans ; Immunohistochemistry ; Keratin-7/metabolism ; Liver Neoplasms/*diagnosis/pathology/radiography/therapy ; Male ; Middle Aged ; Neoplasms, Multiple Primary/*diagnosis/pathology/radiography ; Positron-Emission Tomography ; Tomography, X-Ray Computed

Brugada syndrome can be unmasked by several conditions including a febrile state, marked leukocytosis, and electrolyte disturbances. Herein, we describe a 62-year-old man with cholangiocarcinoma in the first reported case of Brugada syndrome onset following photodynamic therapy.
Bile Duct Neoplasms/*drug therapy ; *Bile Ducts, Intrahepatic ; Brugada Syndrome/diagnosis/*etiology/therapy ; Cardiopulmonary Resuscitation ; Cholangiocarcinoma/*drug therapy ; Electrocardiography ; Fatal Outcome ; Fever/etiology ; Humans ; Klatskin's Tumor/*drug therapy ; Male ; Middle Aged ; Photochemotherapy/*adverse effects ; Predictive Value of Tests ; Treatment Outcome

A 61-year-old male who complained of right upper quadrant pain was referred to the authors for evaluation after his computed tomography suggested biliary adenocarcinoma. The lesion consisted of multiple cysts with papillary mass and peri-ampullay mass. The patient underwent an operation due to a clinical suspicion of biliary cystadenocarcinoma, but the pathology confirmed biliary papillomatosis (BP) after diagnosing intrahepatic papillary neoplasm with high-grade dysplasia and invasive adenocarcinoma with papillary neoplasm from the distal common bile duct to the duodenum. BP is a disease characterized by multiple papillary masses. Its cause has yet to be discovered. It commonly manifests as bile duct dilation but rarely as a ductal cystic change. Under computed tomography or magnetic resonance imaging, both the BP and the cystic neoplasm can show bile duct dilation and a papillary mass, which makes their differential diagnosis difficult. A confirmative diagnosis can be made through a pathologic examination. BP is classified as a benign disease that can become malignant and may recur, though rarely. Its treatment of choice is surgical resection. Laser ablation or photodynamic therapy can be used for unresectable lesions. In the case featured in this paper, biliary papillomatosis was difficult to differentiate from cystic adenocarcinoma due to diffusely scattered multiple large cystic lesions in the liver, and it was histologically confirmed to have become malignant with cystic duct dilation after the operation. This case is reported herein with a literature review.
Adenocarcinoma ; Bile ; Bile Ducts ; Biliary Tract Neoplasms ; Common Bile Duct ; Cystadenocarcinoma ; Cystic Duct ; Diagnosis, Differential ; Dilatation ; Duodenum ; Humans ; Laser Therapy ; Liver ; Magnetic Resonance Imaging ; Male ; Papilloma ; Photochemotherapy

BACKGROUND/AIMS: Hemobilia is a rare cause of upper gastrointestinal bleeding. Endoscopic retrograde cholangiopancreaticography (ERCP) is considered to be an excellent diagnostic and treatment modality. Thirty-seven cases of hemobilia with different underlying pathologies were analyzed to illustrate clinical features and to evaluate the role of endoscopic management. METHODS: A total of 37 patients (26 men and 11 women; mean age, 66.2+/-15.3 years) who were confirmed to have hemobilia by ERCP in a single center from 2000 to 2010 were reviewed retrospectively. Patients with iatrogenic causes of hemobilia were excluded in this study. RESULTS: The causes of hemobilia were hepatocellular carcinoma in 14, bile duct and gallbladder malignancies in 12, common bile duct stones with cholangitis in 4, acute cholecystitis in 4, and pancreatic cancer in 2 patients. The clinical features of hemobilia were jaundice (89.2%), abdominal pain (78.4%), and melena (13.5%). The cholangiographic findings of hemobilia were amorphous filling defects in 15, tubular filling defects in 6, and cast-like filling defects in 6 patients. Endoscopic management included endoscopic nasobiliary drainage in 26 patients and endoscopic retrograde biliary drainage in 7 patients. Biliary obstruction caused by hemobilia was successfully treated with endoscopic biliary drainages in most cases. CONCLUSIONS: The most common non-iatrogenic causes of hemobilia were hepatobiliary malignancies, and the majority of patients presented with jaundice and abdominal pain. Endoscopic biliary drainage is recommended as the initial management to control biliary obstruction.
Abdominal Pain/etiology ; Adult ; Aged ; Aged, 80 and over ; Bile Duct Neoplasms/complications ; Carcinoma, Hepatocellular/complications ; Cholangiopancreatography, Endoscopic Retrograde ; Cholecystectomy ; Cholecystitis/complications ; Drainage ; Female ; Gallstones/complications ; Hemobilia/*diagnosis/etiology/therapy ; Humans ; Jaundice/etiology ; Liver Neoplasms/complications ; Male ; Middle Aged ; Pancreatic Neoplasms/complications ; Retrospective Studies