The 5-year survival rate for patients with ampullary cancer (n=85), gallbladder cancer (n=95), distal cholangiocarcinoma (n=94), perihilar cholangiocarcinoma (n=54), intrahepatic cholangiocarcinoma (n=47), and widespread cholangiocarcinoma (n=17) was 65%, 60%, 52%, 27%, 44%, and 12%, respectively. Patients with ampullary cancer showed favorable survival. In contrast, perihilar cholangiocarcinoma and widespread cholangiocarcinoma were associated with poor survival. Better survival results would be achieved by margin-negative R0 resection for perihilar cholangiocarcinoma or widespread cholangiocarcinoma.

According to the biliary tract cancer registry, accurate diagnosis and surgery with definite lymph node dissection are necessary for improving the treatment outcomes of ampullary region carcinoma. EUS and IDUS are useful for the diagnosis of ampullary carcinoma, and the management is decided according to the result. In the biliary tract cancer clinical guidelines, when recognizing a tumor, pancreaticoduodenectomy is recommended. However, if the tumor does not invade Oddi's muscle layer (T1a), the possibility of lymph node metastasis is extremely low, and the application of papilla resection is also examined. When pancreaticoduodenectomy is performed, it is possible to perform safe surgery that suppresses the amount of bleeding by ligating the IPDA prior to pancreatectomy, and to ensure the dissection of No. 14 lymph node. Laparoscopic pancreaticoduodenectomy can be performed only in cases where lymph node dissection is unnecessary and strict case selection is necessary for the current indication. Robot-assisted laparoscopic pancreatoduodenectomy is effective for delicate procedures such as pancreaticojejunostomy, and hopefully insurance will be covered in the future.

Surgical resection is the only radical treatment for bile duct cancer. However, it is often discovered in an advanced and unresectable state, either because it is advanced cancer at the time of diagnosis or because of extensive invasion or distant metastasis. Chemotherapy is performed after resection or in unresectable cases. In Japan, the standard first-line therapy for unresectable bile duct cancer is combination therapy with gemcitabine plus cisplatin. S-1 is often used as second-line therapy, but evidence for it has not been established, including in an adjuvant chemotherapy for bile duct cancer. Chemotherapy in resectable cases and conversion therapy in unresectable cases is sought through trial and error. The results of currently ongoing prospective trials are awaited. Advances in biomarkers to predict the outcome following chemotherapy and in personalized medicine are also anticipated.

We often experience early plastic stent (PS) occlusion in preoperative endoscopic biliary stenting (EBS) with distal malignant biliary obstruction (DMBO). In our retrospective analysis for preoperative cases with DMBO, which consisted of 22 cases of pancreatic cancer and 14 cases of biliary tract cancer, we did not require re-drainage with the self-expandable metallic stent (SEMS) group. On the other hand, in the PS group, the re-drainage rate was 81.8% and 23.5 % (p=0.008) and the mean stent patency was 32.8 days and 57.6 days (p=0.006) for 7Fr and 8.5Fr, respectively. In the 8.5Fr PS group the stent patency rate was 94.1% on the 25 day. No differences were found between the PS and SEMS groups in regards to duration of surgery, amount of bleeding, hospitalization, and postoperative complications. For preoperative EBS, if the waiting period before surgery is less than 25 days, we should use a PS whose size is more than 8.5Fr, and if it is 25 days or more, we should use SEMS.

Treatment outcomes of biliary intervention using standard and short type single balloon enteroscope (SBE) was compared in patients with surgically altered gastrointestinal anatomy (SAGA). Seventy-three and one hundred ten cases were included in a standard and short group. More cases of Roux-en-Y anastomosis with prior gastrectomy and of malignant biliary obstruction were included in a short group. The success rate of reaching the target site was improved and the time of reaching the target site was shorter in a short group. The success rate of biliary cannulation was also better in a short group, though there were no difference in the success rates of ERCP-related intervention between two groups. The overall success rate was superior and the overall procedure time was shorter in a short group. Our study showed that the treatment outcomes were improved using a short type SBE when performing biliary intervention in patients with SAGA.

Primary biliary cholangitis (PBC) is a chronic cholestatic liver disease, but pathophysiology of this disease has not been clarified yet. However, recent studies on disease susceptible genes and immunological mechanisms of cholangiocyte destruction are unveiling the cause of this disease. A new classification of histopathological stages and activities was established, and the so-called 'PBC/AIH overlap syndrome' has come to be classified as a PBC with high activity of hepatitis. A new class medicine, obeticholic acid, has been used in the United States since 2016, but clinical usability of this medicine is unknown because of frequent side-effect of pruritus and tremendously high cost. In this situation, the usefulness of bezafibrate that has been used as a second-line medicine for more than 10 years in Japan has come to be recognized recently in western countries. In addition, clinical trials on immunotherapies are also ongoing. New scoring systems to predict outcomes of patients with PBC (GLOBE and UK-PBC scores) were established recently and the calculators are freely available on website.

Primary sclerosing cholangitis (PSC) is a chronic cholestatic disease of unknown aetiology, which is characterized by the progressive stenosis and destruction of the bile ducts due to diffuse inflammation and fibrosis, and which eventually results in cirrhosis and liver failure. Inflammatory bowel diseases (IBD) are frequently noticed as comorbidities and inflammation at intestinal mucosa and abnormal bacterial flora are supposed to be involved with etiology of PSC. Onion skin lesion is a characteristic histological feature but rare to be seen. Therefore cholangiographic findings including beaded appearance and pruned tree appearance is diagnostic. Unfortunately, despite the severity of this disease, no standardized medical treatment is available currently, and although autoimmune reactions are believed to play a role in PSC pathogenesis, immunosuppressive therapy has not been effective. As a result, the long-term outcome for patients with PSC is unfavourable and is characterized by a high mortality rate, with liver transplantation (LT) being the only option for patients with advanced PSC.

We report a case of a 65-year-old man who underwent laparoscopic cholecystectomy for chronic cholecystitis and cholelithiasis 16 years before admission. The patient was presented to our department because of recurrent abdominal pain. CT scan revealed a metallic density artefact in the lower bile duct. Clip migration into common bile duct was confirmedby ERC, and the clip migration was accompanied with stone formation. The patient was successfully managed by endoscopic stone and clip removal.

Laparoscopic cholecystectomy is a gold standard surgery for cholecystolithiasis, however, endoclip migration and stone formation have been reported in late postoperative years. We report a case of clip migration into the bile duct accompanied with stone formation16 years after laparoscopic cholecystectomy.

A 74-year-old woman underwent subtotal stomach-preserving pancreaticoduodenectomy for distal cholangiocarcinoma. The postoperative histopathological diagnosis was Bd tub1 T3a N1 (#17b) M0 DM0 HM0 EM0 PV0 A0 R0 stage IIB (General Rules for Clinical and Pathological Studies on Cancer of the Biliary Tract, 6^th Edition). Adjuvant chemotherapy with TS-1 80 mg/m² (14-day course, 7 days between courses) was administered for 1 year. Four years and 8 months later, an elevated CA19-9 result (67U/mL, range: 0-37U/mL) prompted further tests. The test results indicated primary intrahepatic cholangiocarcinoma; therefore, surgery was planned. Laparotomy was performed at the site of the previous Benz incision. The right lobe was turned and S7 partial hepatectomy was performed without using the Pringle maneuver.

Although this hepatectomy followed a pancreaticoduodenectomy, it was performed safely. The postoperative course was good and the patient was discharged 8 days after surgery. The resected liver (S7) tumor was 0.7cm and diagnosed with Well differentiated Tubular adenocarcinoma T1 N0 M0 R0 stage I (General Rules for Clinical and Pathological Study of Primary liver Cancer The 6^th Edition) histopathologically.

The patient remains relapse-free and is being seen on an outpatient basis.

Therefore, it is important to perform hepatectomy as the initial therapy.

Heterochronic intrahepatic cholangiocarcinoma after distal cholangiocarcinoma resection is uncommon, with only 7 reported, including the present case. Despite the pathologic diagnosis, long-term survival is possible because resection could be performed.

A 74-year-old man with abdominal pain was referred to our hospital. He received a curative cholecystectomy for an early-stage gallbladder carcinoma (pT1bN0M0, pStage I) 14 years earlier. Imaging studies detected an advanced common bile duct cancer along with pancreaticobiliary maljunction without biliary dilatation. The patient underwent a pylorus-preserving pancreaticoduodenectomy.

There have been 12 reported cases of pancreaticobiliary maljunction without bile duct dilatation, including our case, in which bile duct carcinoma developed following cholecystectomy. Comparison of cases indicated that the time period from cholecystectomy until the occurrence of bile duct carcinoma was shorter in 7 patients who had received a cholecystectomy for gallbladder carcinoma (5.4 years) than those with benign gallbladder lesions (19.5 years). Although no consensus has yet been reached about the best surgical treatment for patients with pancreaticobiliary maljunction without bile duct dilatation, careful postoperative long-term follow-up should be required in cases undergoing cholecystectomy for gallbladder carcinoma because of a possible occurrence of metachronous bile duct carcinoma.

A 64-year-old man was admitted to our hospital for investigation of jaundice and liver function abnormalities. Abdominal computed tomography (CT) revealed intrahepatic duct dilatation due to tumor at the middle of bile duct of 2cm from the confluence of hepatic ducts. The bile cytology showed adenocarcinoma. Subtotal stomach-preserving pancreatoduodenectomy and extended left hepatectomy with regional lymph node dissection was performed. Pathological examination showed neuroendocrine tumor (NEC) with concurrent adenocarcinoma of the bile duct, finally diagnosed as mixed adenoneuroendocrine carcinoma (MANEC) in accordance with the 2010 WHO Classification of Tumors of the Digestive System. The patient had liver metastases, 6 months after the operation. We started chemotherapy with Gemcitabine and CDDP, but liver metastases progressed, dying 15 months postoperatively. MANEC of the bile duct is poor prognosis. Therefore, multidisciplinary treatment including surgery and chemotherapy must be developed.

A 36-year-old woman was admitted to our hospital associated with BTD. An abdominal CT and MRCP demonstrated severe BTD. Preoperative radiographic examinations could not detect ABV. Preoperative diagnosis was type IVA of congenital BTD. At the time of operation, a cystic artery was running on the ventral side of the common hepatic duct. Two tubular cords were recognized from hilus hepatis to posterior side of common bile duct. An intraoperative cholangiography revealed the aberrant posterior hepatic duct and caudate branch. The holes of these two bile ducts were connected into one hole. Main and variant bile ducts were separately anastomosed to the jejunum. Her postoperative course was uneventful. There are no previous reports in the literature of the presence of congenital CBTD and rare ABV like this case. This case was regarded as the warning-like case when performing an operation

Hemobilia after endoscopic retrograde cholangiopancreatography (ERCP) is rare, but some severe and fatal hemorrhages have been reported. We recently experienced a case of severe hemobilia and cholangitis after endoscopic biliary plastic stent insertion for the common bile duct stone. The second endoscopy revealed blood spurting from the papilla of Vater. Laboratory data showed anemia and inflammatory findings. Subsequent Computed tomography (CT) showed high density materials in the common bile duct. But there are no lesion of pseudoaneurysm by CT and interventional angiography. Therefore the emergency laparotomy was indicated under a preoperative diagnosis of progressive hemobilia and cholangitis. The intraoperative cholangioscopy showed bleeding and laceration of bile duct. Although late biliary stricture was occurred, the patient recovered after endoscopic balloon dilatation. We describe a rare case of hemobilia after endoscopic biliary plastic stent insertion.

We report successful percutaneous transhepatic retrograde biliary drainage (PTRBD) in a patient with a biliary fistula following hepatic resection, after endoscopic retrograde biliary drainage (ERBD) proved difficult. A 71-year-old man presented with a postoperative biliary fistula with abscess formation following extended left lobectomy for hepatocellular carcinoma, and was diagnosed with an isolated biliary fistula caused by stenosis of the posterior sector bile ducts. We attempted stent placement, but ERBD was difficult following total gastrectomy; percutaneous transhepatic biliary drainage (PTBD) was unsuccessful. We inserted a catheter through the abscess cavity to the terminus of the right posterior bile duct (B6a). Using a relatively stiff guidewire, we perforated the peripheral wall of the bile duct and hepatic parenchyma, advancing to the abdominal cavity inferior to the right diaphragm. We extracted the guidewire, leaving an indwelling catheter in the bile duct. This technique can be considered as a PTRBD variant.

This case is of a 42-years-old man with a history of surgery for congenital biliary dilatation. Two years after surgery, a 25-mm mass was indicated at the intrapancreatic bile duct or residual cyst on computed tomography (CT) and magnetic resonance imaging (MRI). After confirming a diagnosis of residual bile duct or cyst carcinoma, we performed surgical resection. Pathological examination indicated the presence of neuroendocrine carcinoma (NEC) and tubular adenocarcinoma components in the mass, each occupying 70% and 30% of the mass, respectively. Based on these findings, the final diagnosis was MANEC at the residual cyst. The patient remains alive more than 5 months after diagnosis, without adjuvant therapy. According to previous reports, MANEC of the bile duct is a disease with poor prognosis. Moreover, carcinoma of the residual bile duct/cyst also exhibits poor prognosis. Therefore, surgical treatment for congenital biliary tract dilatation should not leave a cyst. This is the rare case report of MANEC with congenital biliary dilatation.