Patients with carcinoma of the papilla of Vater have a more favorable prognosis after curative resection than those with ductal carcinoma of the pancreatic head or distal bile duct. However, even after apparently curative resection, some of the patients with carcinoma of the papilla of Vater will experience the local recurrence as well as the distant metastasis, and nodal status is considered to be one of the most important prognostic factors. Therefore, lymph node dissection is an important component of curative surgery, especially in case of advanced carcinoma of the papilla of Vater which apparently invades the pancreatic parenchyma. In this article, I will focus on the clinical significance of the exact D2 lymph node dissection based on the clinical anatomy, including lymph nodes around the superior mesenteric artery and around the root of the small-bowel mesentery.

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Biliary tract cancers are low-radiosensitive adenocarcinomas and occur in deep-seated organs. Furthermore, normal tissues with higher radiosensitivity exist contiguously with the diseased sites. For these reasons, radiation therapy has not been considered as a good indication for these cancers.
Since the prolonged survival achieved by 30- to 60-Gy external beam radiation therapy was reported in the 1970s, in addition to intraoperative radiation therapy, postoperative radiation therapy, and intraluminal brachytherapy, combinations of external radiation therapy and chemotherapy have come into use.
The purpose of radiation therapy for unresectable biliary tract cancer is to prolonged survival or prolonged stent patency, and to provide palliation of pain. For unresectable bile duct cancer, there are a number of studies showing that radiation therapy is superior to the best supportive care.
As radiation therapy, external-beam radiation therapy is usually performed, but combined use of intraluminal brachytherapy with external beam radiotherapy is more useful for making the treatment more effective.
A number of studies show the usefulness of radiation therapy for biliary tract cancer, but most of these studies are small prospective or retrospective studies. Thus, radiation therapy is not a standard treatment in biliary tract cancer. Implementation of large multi-institional RCTs is desired.

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In biliary tract cancer, systemic chemotherapy should be indicated for unresectable disease, and patients with good performance status have the survival benefit of chemotherapy. Recently, phase II studies of gemcitabine and tegafur/gimeracil/oteracil potassium (S-1) were conducted in Japan, and they showed promising activity against advanced biliary tract cancer. As a result, gemcitabine and S-1 have been approved for biliary tract cancer. Based on retrospective analyses and pooled analyses of phase II trials, gemcitabine and cisplatin are expected to be effective for unresectable biliary tract cancer. Furthermore, combination chemotherapy are being investigated, and randomized clinical trials between monotherapy using gemcitabine or S-1 vs. combination chemotherapy, e.g., gemcitabine+cisplatin or gemcitabine+S-1, has been under investigation. In foreign countries, combination of gemcitabine and cisplatin or gemcitabine and capecitabin. As adjuvant therapy after surgery, gemcitabine or capecitabine is applied in randomized clinical trials. Based on some randomized clinical trials, a standard chemotherapy will be established in biliary tract cancer soon.

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In cases underwent cholecystectomy, we was measured amylase concentration of the bile and examined clinically and histologically. This study was the object for seven patients with over 1,000 IU/L (high amylase group) and seven patients in less 100 IU/L (control group) in amylase concentration of the bile. In high amylase group, all cases showed maljunction of the pancreatobiliary or a long common channel, and the gallbladder mucosa in the most of those showed papillary type and positive in Ki65LI and MAC5AC. In the high amylase group, the findings on the preoperative US or EUS showed more wall thickness. We suggest that the gallbladder mucosa with high amylase concentration of the bile is high risk of gallbladder cancer and that mucosal thickness of gallbladder on US is important finding.

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We reviewed the maximal blood flow velocity (Vmax) in the gallbladder wall (cystic artery) using Doppler ultrasonography in 46 patients (cancer N=14, benign N=32). The Vmax of cancer (45.0±13.2 cm/s) was significantly higher than that observed for chronic cholecystitis, polyps or ADM (adenomyomatosis) (p<0.01). An ROC analysis showed a Vmax cut-off value of 35.2 cm/s, while the sensitivity was 85.7% and the specificity was 88.2%. The histological findings clarified the fibrous hyperplastic change in the intraarterial membrane of the basal area lying under the cancer nest. This alteration was not found in the non-cancer areas, polyps or ADM. These results suggested that stenotic change of the artery may cause an increase in the blood flow velocity. In conclusion, a blood flow analysis makes it possible to directly evaluate cancerous changes in the preoperative examination.

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This is a brief review of the regulatory mechanisms and abnormalities of bilirubin transport and metabolism in hepatocytes. The induction and suppression of hepatocyte transporters by nuclear receptors are outlined. A linkage has been recognized between UGT1A1 polymorphism (UGT1A1^*6) that causes Gilbert's syndrome and UGT1A polymorphisms (UGT1A6^*2 and UGT1A7^*3) that cause disturbance of drug glucuronide conjugation and high incidence of lung cancer. Defects of MRP2 in the Dubin-Johnson syndrome and of GST-alpha in the Rotor's syndrome also indicate disturbance in the metabolism of anionic drugs. Hepatic enzymes and transporters are also reduced in acquired hepatobiliary disorders that cause jaundice; thus, occurrence of abnormalities in the metabolism of anionic drugs should be also taken into consideration in acquired hepatobiliary diseases with jaundice.

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The patient was a 75-year-old woman who was referred to our hospital for detailed examination of liver dysfunction. Abdominal CT and EUS revealed a mass lesion in the distal bile duct, while ERC showed a filling defect. The patient was preoperatively diagnosed with papillary distal bile duct carcinoma, and surgical resection was performed. Macroscopic findings for the resected specimen included a papillary tumor presenting with redness in the distal bile duct. Histologically, mildly atypical gland and hyperplasia of muscle fibers and fibrous tissue were observed. Based on these findings, a final diagnosis of adenomyomatous hyperplasia was made. Benign tumors rarely occur in the bile duct, and among them, adenomyomatous hyperplasia is particularly rare. As no characteristic findings on diagnostic imaging have been elucidated for this disease, accurate preoperative diagnosis is currently difficult. Detailed investigation through accumulation of cases is necessary in the future.

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Bile leakage after hepato-biliary-pancreatic surgery is one of the major complications. However, a definitive management strategy for postoperative bile leakage remains elusive. We experienced four cases of postoperative bile leak managed by biliary ablation with absolute ethanol between January 2000 and December 2006. Of four patients, one patient underwent living donor liver transplantation (recipient), two patients underwent hepatectomy, and one patient underwent pancreaticoduodenectomy. Biliary fistula had no communication with the remainder of the biliary tree or alimentary canal in all patients, therefore, we performed biliary ablation with absolute ethanol for isolated biliary fistula. Biliary ablation was succeeded in all patients without any complications. Biliary ablation is an effective interventional treatment for refractory isolated biliary fistula after surgery.

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A case of inflammatory polyp of the gallbladder accompanied localized Adenomyomatosis is reported. A 72-year-old woman who was pointed out having 2 gallbladder polyps was admitted to the hospital. Abdominal ultrasonography (US) and Computed tomography (CT) showed a polyp on adenomyomatosis at the fundus, a semi pedunculated polyp at the body of the gallbladder. Endoscopic ultrasonography (EUS) showed that the polyp at the fundus was pedunculated. A laparoscopic cholecystectomy was carried out. Histopathological studies of resected gallbladder indicated that the lesion was inflammatory polyp. We thought that the mechanism of development was highly suggestive.

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A 66-year-old man who had undergone biliary diversion for congenital biliary dilatation of the bile duct at the age of 46 years was admitted to our hospital, after developing a liver abscess complicating intrahepatic stones. After cholagioscopic lithotripsy, percutaneous transhepatic cholangioscopy disclosed a reddish, nodular lesion, 7 mm in diameter, in the right hepatic duct. Pathologic examination of biopsy specimen from the lesion indicated moderately differentiated adenocarcinoma. Because the patient had chronic hepatitis B and severe liver dysfunction, surgical treatment was not pursued. Brachytherapy using a remote after-loading system was not effective. Microwave coagulation therapy achieved satisfactory local control. This patient's course illustrates the risk of intrahepatic stones and cholangiocarcinoma after surgical treatment of congenital dilation of the bile duct; long-term follow-up is required. Here, percutaneous transhepatic cholangioscopy detected a cholangiocarcinoma at a relatively early stage.

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We report the case of a patient with pancreaticobiliary maljunction with a long common channel of the duct of Wirsung origin which presented with choledocholithiasis with acute pancreatitis. A 38-year-old man was admitted with right upper abdominal pain and high fever. The diagnosis of choledocholithiasis with acute pancreatitis was made. ERCP depicted a pancreaticobiliary maljunction with a short communicating duct between the upper border of the long common channel and the duct of Santorini. The extrahepatic bile duct was dilated with a diameter of 12 mm. Small pancreatic ductal branches entered into the long common channel. He underwent a cholecystectomy with resection of the extrahepatic bile duct, followed by a hepaticoduodenostomy. The above ERCP findings suggest that the long common channel in the current case originated from the duct of Wirsung.

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We report a case of cancer of the ampulla of Vater presenting as a polyp type. A 75-year-old woman had undergone distal gastrectomy with B-I reconstruction for advanced gastric cancer. During follow-up, bile duct dilatation was pointed out by abdominal CT, and gastroduodenoscopy showed a polyp lesion of the ampulla of Vater 32×31 mm in size. She had no clinical symptom and the laboratory data revealed mild liver dysfunction and increased serum DUPAN-2 level. Histological examination of biopsy of the ampullary lesion revealed a well differentiated tubular adenocarcinoma, and she underwent pancreatoduodenectomy on January, 2007. Pathological examination of resected specimen showed cancer invasion reaching to the duodenal muscle (JSBS classification; pT3 pN0 M0 fStage III). Ampullary cancer with polyp type is very rare, which is reported just to be 1.1% of all ampullary cancer from the reports of the cancer accumulation by Japanese Society of Biliary Surgery. Further case accumulation is required to establish the appropriate treatment plan (including endoscopic resection or operative method) for polyp type ampullary cancer.

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A 57-year old-man was admitted to our hospital with obstructive jaundice. Abdominal multi-detecter row CT (MDCT) demonstrated a biliary tumor at the hepatic confluence with intrahepatic bile duct dilatation. The right posterior portal vein was first ramified from the main portal trunk. The portal bifurcation of the left and right anterior portal veins was possibly involved by the tumor. Cholangiography showed a stricture of the extrahepatic bile duct from the hepatic hilus to the lower bile duct. Considering the residual liver volume and the anatomical variation of the portal vein, left trisectionectomy with caudate lobectomy plus pylorus preserving pancreatoduodenectomy was performed. Histologically, diffusely spreading bile duct cancer invaded the liver and pancreas. Resectional procedure should be determined considering both operative radicality and risk.

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Torsion of the gallbladder is a relatively rare cause of an acute abdomen, and its diagnosis prior to operation has been difficult. We report a case of torsion of the gallbladder. Patient was thin elder female, over 90 years old. She was admitted to our hospital, diagnosed as having acute cholecystitis. An abdominal ultrasound and CT showed an extremely swollen gallbladder with thickened wall. MRCP did not depict the neck of the gallbladder. Patient underwent emergency open cholecystectomy. Operative finding showed torsion of the gallbladder with a 360-degrees volvulus, so-called a complete type. Gallbladder was fallen into the gangrenous cholecystitis state. The patient had uneventful postoperative course and was discharged from our hospital. Since torsion of the gallbladder is usually caused by the floating gallbladder, emergency laparoscopic cholecystectomy is recommended because of its anatomical character. For the preoperative diagnosis and precise treatment, it is important to keep the anatomical and imaging feature of this disease in mind.

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Ultrasonography, including color Doppler US is useful in assessing right hepatic artery invasion and portal vein invasion of the superior bile duct carcinoma. On the other hand, endoscopic ultrasonography via the transduolenal scanning has some limitations in staging superior bile duct carcinoma compared to it of inferior bile duct carcinoma. Intraductal ultrasonography produces high-quality cross-sectional images of the bile duct without blind area, and is useful in assessing tumor depth infiltration, hepatic parenchymal invasion, portal vein invasion, and right hepatic artery invasion of superior bile duct carcinoma. However, it has limitations in assessing lymph nodes metastases. The assessment of longitudinal cancer extension along the bile duct is a promising aspect of this area. Transpapillary IDUS prior to biliary drainage is essential to prevent inflammatory changes that may result from mechanical irritation by a biliary drainage catheter.

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MDCT is a reliable tool for the diagnosis of hilar cholangiocarcinoma. Primary tumor is appeared as focal wall thickening and/or intraductal mass-formation accompanied with enhancement of contrast medium. To reveal the tumor extension and decide the proper biliary drainage root, MDCT examination had better be done prior to biliary drainage. Original thin-axial images are important to precise analysis of bile duct anatomy and tumor extension assumed by reconstructed 3-dimensional images. Analysis of biliary anatomy should be performed based on that of portal vein. To make full use of MDCT, picture archiving and communication system is necessary.

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