Congenital biliary dilatation (CBD) is a congenital malformation involving both extrahepatic bile duct dilatation and pancreaticobiliary maljunction (PBM). Although pathogenesis of bile duct dilatation is unknown, PBM causes reciprocal reflux between the pancreatic juice and bile and results in various biliary and pancreatic pathologies. For a diagnosis of CBD, both abnormal dilatation of the bile duct and PBM must be evident. Bile duct dilatation should be diagnosed based on age-related limits on the maximum diameter of the common bile duct using diagnostic imaging (e.g., ultrasonography, magnetic resonance cholangiopancreatography, and multiplanar reconstruction imaging by multidetector row computed tomography). Endoscopic retrograde cholangiopancreatography, percutaneous transhepatic cholangiopancreatography, and operative cholangiography would be avoided as bile duct measurement tools. Typical concomitant anatomical characteristics of extra- and intra-hepatic bile ducts should be also considered when diagnosing CBD. Diagnosis of PBM, an abnormally long common channel, and/or an abnormal union between the pancreatic and bile ducts must be established by various radiological imaging.

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Objectives: The administration of gemcitabine (Gem) as postoperative adjuvant chemotherapy for biliary tract cancer was examined in consideration of tolerability. Method: Thirty cases of resected biliary tract cancer are randomly assigned to either a standard schedule or a bi-weekly schedule of gemcitabine. The primary endpoint was the compliance rate for 6 courses, and the secondary end-point was 3-year overall survival time, disease-free survival and adverse events. Results: Dose Intensity was significantly high and adverse events was more frequent in the standard group. But the compliance rate, the mean overall survival time and the disease free time were not different in these two groups. Conclusion: It was suggested that the bi-weekly schedule could be considered a choice for the patients who had difficulty with the standard schedule of gemcitabine.

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We examined the clinical efficacy of endoscopic treatment for the patients aged 75 and over with common bile duct stones (CBDS). 340 patients who were performed ERCP for choledocholithiasis were recruited. There were no significant differences in the treatment efficacy, the complications, and the recurrence of CBDS between the two groups. However, the recurrence rate of CBDS after resection of gallbladder with stones were 3.6% and 11.8% in Group A and Group B, respectively (p=0.02). Regarding elderly patients, endoscopic treatment for CBDS should be performed under strict risk management, and in these conditions, it is effective and safe.

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The operative procedure for perihilar cholangiocarcinoma (PHC) should be selected not only by the longitudinal tumor extension to the bile duct and tumor invasion to the major hilar vessels, but also functional reserve of the future remnant liver. Right trisectionectomy (RTS) is the most extended resection among the standard resectional procedures (RTS, right hemihepatectomy, left trisectionectomy (LTS) and left hemihepatectomy). Despite the recent advance in surgical techniques and perioperative patient care, RTS is still considered to be associated with high mortality due to hepatic failure. Meanwhile, LTS is recently performed more frequently in patients with Bismuth type IV tumors of left side predominance in a specialized center. LTS can produce a longer resection margin for the right posterior sectional bile duct. However, LTH may be technically demanding with association to the high postoperative morbidity. Both hepatic trisectionectomy for PHC may be highly skillful and difficult procedures among the hepatobiliary surgeries. For successful surgery, careful evaluation of 3D hilar anatomy and also the functional reserve of future remnant are crucial. Herein, clinical significance of RTS and LTS for PHC is discussed, as well as the important 3D anatomical variations for these procedures.

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Although laparoscopic surgery for gastrointestinal disease has been recently performed worldwide, the indication of laparoscopic surgery for biliary tract disease is limited except laparoscopic cholecystectomy and laparoscopic common bile duct exploration. Recently, the feasibility of laparoscopic surgery for choledochal cysts and biliary tract cancer has been reported from some institutions. However, it remains challenging because of technical difficulties. Laparoscopic approach is also difficult for patients with preoperative cholecystitis and cholangitis. Further discussion is needed to safely perform laparoscopic surgery before the indication is widened in each biliary tract disease. We herein review recent reports and describe future prospects of laparoscopic surgery for biliary tract disease.

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We report a rare case of primary liver adenosquamous carcinoma. The 79-year-old male patient had a 6.5cm liver tumor with almost clear borders localized by abdominal CT at the medial segment and anterior segment near ventral side. The tumor border was slightly enhanced, and the inside was slightly enhanced and heterogeneous. Necrosis and liquefaction were suspected in part of the tumor by MRI. Extended left hepatectomy associated with portal vein resection was performed under a diagnosis of intrahepatic cholangiocarcinoma. The border of the resected tumor tissue and circumference were comparatively clear. The inside of the tumor was solid and gray-white, but fragile and partly collapsed. The tumor was diagnosed as adenosquamous carcinoma. The patient died without recurrence at 3 years 3 months after surgery. Because this tumor enlarged rapidly and had central necrosis, enhancement at the tumor border and internal necrotic change are important findings indicative of adenosquamous carcinoma.

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A 74-year-old woman was admitted to our hospital with right upper quadrant pain after dinner. Blood tests revealed elevated pancreatic enzymes, however, an abdominal CT scan showed ascites in Morrison's pouch and no significant evidence for presence of pancreatitis or cholelithiasis. Although abdominal paracentesis showed no evidence of bile leak, we could not deny the possibility of biliary peritonitis. We performed endoscopic nasobiliary drainage (ENBD) and confirmed the improvement of her general condition. To investigate whether there is a perforation in the gallbladder, endoscopic nasogallbladder drainage (ENGBD) was performed. A leakage from the gallbladder was confirmed by ENGBD. Open cholecystectomy was performed with the preoperative diagnosis of idiopathic perforation of gallbladder. Histopathological examination showed a mild inflammation at the site of perforation without thrombosis. Here we report the first case of idiopathic perforation of gallbladder diagnosed by ENGBD.

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A 72-year-old man was hospitalized for acute cholecystitis, and conservative therapy was started. On the fourth disease day, however, anemia progression and fluid collection in Morrison's pouch were observed. Therefore, emergency surgery was performed. Laparotomy findings included intra-abdominal hemorrhage due to oozing from the gallbladder wall attached to the liver. There was no histopathological evidence of gallbladder perforation. The oozing was considered to be attributable to weakening of blood vessels in the gallbladder wall due to aging, along with increased internal pressure caused by gallbladder tension arising from the outflow of intra-cholecystic hemorrhage to the bile duct being prevented by intra-cholecystic hematoma. Gallbladder hemorrhage and hemorrhagic cholecystitis are rare. However, we should always consider these diseases because they can lead to serious clinical conditions such as shock. We presented a rare case of hemorrhagic cholecystitis with intra-abdominal hemorrhage without gallbladder perforation with a discussion of the relevant literature.

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A 49-year-old man was admitted for abdominal pain and nausea. He had been treated with hemodialysis for chronic renal failure. Contrast-enhanced abdominal computed tomography (CT) revealed fluid collection between hepatic hilar and pancreas head, common bile duct stones, atherosclerosis, gallstones, and multiple renal cysts. Liver dysfunction was slight on blood examination. On 2nd hospital day, we placed biliary stent endoscopically. On 3rd hospital day, CT revealed ascites. By reviewing the cholagiography on 2nd hospital day, a biliary fistula was recognized just under the junction of the cystic duct. Surgical treatment was performed with cholecystectomy and biliary stone extraction with cholangioscope through the cystic duct on 10th hospital day. T-tube was placed through the fistula. On 35th hospital day, the T-tube was removed and the patient was discharge on 36th hospital day.

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The patient was 66-year-old female. Wall thickness and debris of the gallbladder was detected with the opportunity of medical checkup, and the patient was referred to our hospital. A pancreatobiliary maljunction was recognized by endoscopic ultrasonography (EUS). Endoscopic retrograde cholangiography (ERC) revealed the non-dilated choledochus with slightly irregular margin and an elevated lesion at the fundus of gallbladder. Transpapillary forceps biopsy was performed to investigate the bile duct and endoscopic naso-gallbladder drainage (ENGBD) was placed for the purpose of cytology. Biliary biopsy and bile cytology demonstrated adenocarcinoma and suspected adenocarcinoma, respectively. Preoperative diagnosis was double cancers of the gallbladder and bile duct associated with pancreatobiliary maljunction. Pancreatoduodenectomy was performed. Histology of the resected gallbladder showed diffusely extended intramucosal carcinoma, including the elevated lesion at the fundus, and limited micro areas of subserosa invasion. Intramucosal carcinoma was also recognized at the lower bile duct, 35mm in size. Vessels permeation and lymph node metastasis was not recognized. The patient was free from recurrence for more than five years after the surgery. In cases with pancreatobiliary maljunction, we should note the risk of multiple or occult cancers in the biliary tract, and presurgical investigations must be performed carefully including pathological examinations.

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A 76-year-old woman presented with fever and jaundice. The lower bile duct exhibited enhancement and stenosis on abdominal CT and stenosis on ERCP. Endoscopic transpapillary biopsy was performed, and histopathological evaluation of the specimen revealed poorly differentiated adenocarcinoma containing an invasive micropapillary carcinoma component. As the patient was inoperable due to dementia, she underwent endoscopic biliary stenting with a plastic stent and received follow-up care as an outpatient. Sixteen months after diagnosis, she presented with duodenal invasion of the bile duct carcinoma and pseudoaneurysmal bleeding. Endoscopic hemostasis and duodenal stent placement were performed and she was able to resume oral intake. Eighteen months after diagnosis, she died of the disease. Microscopic examination of the autopsy specimen revealed bile duct adenocarcinoma consisting mainly of an invasive micropapillary carcinoma component, with pancreatic and duodenal invasion and peripancreatic lymph node metastasis. We report a rare case of bile duct adenocarcinoma containing an invasive micropapillary carcinoma component.

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A 62-year-old woman came into our emergency room complaining of convulsions of the left extremity. A contrast-enhanced computed tomography (CT) scan of the brain revealed a 3-cm heterogeneous right parietal lobe mass. Using brain tumor excision surgery, we diagnosed her brain tumor as metastatic adenocarcinoma. A CT scan, echogram, magnetic resonance imaging (MRI) of the abdomen revealed irregular thickening of the gallbladder wall, and an FDG-PET demonstrated that the gallbladder wall had a high-uptake structure. A specimen obtained by percutaneous transhepatic gallbladder fine needle aspiration included cells strongly suspected of adenocarcinoma. No other primary site was detected, so we finally diagnosed her illness as an adenocarcinoma of the gallbladder with solitary brain metastasis. A treatment course of tegafur, gimeracil, and oteracil potassium (S1) was started. After several months, brain tumor recurrence and a primary tumor invasion of the liver were observed. A convulsion attack also occurred. Though palliative radiation therapy to the brain was started, she died 14 months after her first admission. In the gallbladder cancer, it is very unusual to show a sign derived from brain metastasis as an initial manifestation. We report this rare case with a review of the literature.

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Little is known about the clinical aspect of carcinosarcoma of biliary tract. We present a rare case of this tumor followed by a review of the literature. A 73-year-old man visited our department with the chief complaint of jaundice and general malaise. A cholangiography revealed a 20-mm long stenosis in the common hepatic duct. Although bile cytology results indicated class II, cholangiocarcinoma in the perihilar region was strongly suspected. Therefore, pylorus-preserving pancreaticoduodenectomy was performed. A resected sample had a 15-mm tuberous tumour, and HE staining revealed distinct regions of carcinomatous and sarcomatous components as well as a region where both components intermingle. Immunohistochemical examination showed that vimentin and alpha-smooth muscle actin were negative in the carcinoma component, epithelial membrane antigen was negative and pancytokeratin was partially positive in the sarcomatous component. Based on these findings, this case was diagnosed as 'so-called carcinosarcoma' of bile duct origin. The patient died of this disease eight months after the operation.

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An 80-year-old Japanese man had been hospitalized for acute cholangitis approx. 2 yrs prior to his admission to our institution. At that time, the use of intraductal ultrasonography (IDUS) during endoscopic retrograde cholangiopancreatography (ERCP) revealed common bile duct stones; they were removed during endoscopic therapy. The wall thickness of the bile duct was pointed out and then diagnosed as inflammatory wall thickness. The patient was subsequently hospitalized again for acute cholangitis, and at that time ERCP did not reveal stones or abnormality suggesting a neoplasm, but IDUS revealed 5-mm stones and diffuse wall thickness of the common bile duct (the max. thickness was 7mm). This was diagnosed as bile duct carcinoma by biopsy. The patient underwent a pancreaticoduodenectomy and extrahepatic bile duct resection. It was thought that the invasion depth was within the fiber muscular layer, but not all of the cancer was removed because it extended to intrahepatic bile ducts. This case indicates that clinicians should be aware of the potential existence of bile duct carcinoma during treatments for bile duct stones and should perform IDUS. If there is any abnormality in the bile duct wall, further biopsies should be performed.

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A 47-year-old man who had worked in the printing industry for twelve years presented to the hospital with severe epigastralgia. After clinical evaluations, a tumor was found in his liver. He was then referred to our institution, where he was diagnosed with intrahepatic cholangiocarcinoma and was underwent chemotherapy. Nevertheless, fever and nausea were apperared, he was performed hepatic trisegmentectomy. Despite the treatment, the patient's intrahepatic cholangiocarcinoma progressed, and he died from tumor-related complications. The resected specimen showed two intrahepatic cholangiocarcinomas in S6 and S8/4, and biliary intraepithelial neoplasia extending to the intrahepatic bile ducts between these two tumors. Herein, we summarize the findings of this case experienced 20 years ago and a review of the literature.

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We report a case of clear cell carcinoma of cystic duct in a 78-year-old man, who was referred to our hospital with the complaint of severe obstructive jaundice. Abdominal CT scan showed intrahepatic bile duct dilatation and a papillary tumor in the extrahepatic bile duct and the cystic duct. Since microscopic examination of the biopsy specimen showed poorly differentiated adenocarcinoma, we performed extrahepatic bile duct resection and regional lymph node dissection. Pathological examination revealed that most of the tumor cells had large clear cytoplasm and we diagnosed clear cell carcinoma of cystic duct. Ten months after operation, liver metastases were detected. After chemotherapy (GEM+CDDP), we performed liver resection for metastatic clear cell carcinoma. A case of primary clear cell carcinoma of the cystic duct is extremely rare.

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A 46-year-old woman was diagnosed with a 15-cm cystic hepatic tumor in the medial and anterior segment of the liver in 1997, and cystic drainage and minocycline injection therapy had been performed repeatedly at another hospital. She was referred to our hospital for surgical treatment of abdominal distension in 2008. Abdominal computed tomography revealed an enlarged multilocular cystic lesion with calcification, and also revealed enhancement of a thickened wall on intravenous contrast agent administration. Hepatobiliary cystadenoma or cystadenocarcinoma was diagnosed, and central hepatic bisegmentectomy was performed. The resected specimen contained the multilocular cystic tumor, and this tumor was histopathologically diagnosed as a mucinous cystic neoplasm (MCN) of the liver with ovarian-like stroma without malignant components. Estrogen and progesterone receptors were identified by immunohistological examination. There is no evidence of tumor recurrence after surgery till 2015. Although MCN of the liver comprises a wide range of histopathologic atypia, complete resection provides favorable prognosis. MCN of the liver should be considered as a differential diagnosis for simple hepatic cysts, because some patients with MCN of the liver might be incorrectly treated for a simple hepatic cyst.

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A standard treatment for ampullary region carcinoma is pancreaticoduodenectomy with regional lymph node dissection, because some patients have lymph node metastasis once the carcinomas invade the sphincter of Oddi. Overall 5-year survival rate after pancreaticoduodenectomy exceeds 90% when it is ampullary region carcinoma of Stage 0-IB. Lymph node metastasis is the strongest prognostic factor, and, besides, pancreatic invasion, histological differentiation and lymphovascular infiltration are reported to be significant worse prognosic factors. Local resection such as transduodenal papillectomy is less invasive and safer than pancreaticoduodenectomy and, theoretically, this procedure could be indicaed for ampullary region carcinoma without invasion to the sphincter of Oddi. However, since accurate preoperative diagnosis of the invasion to the sphincter of Oddi is currently difficult, local resection should not be indicated for patients with preoperative proven carcinoma unless pancreaticoduodenectomy is not feasible. For benign ampullary region neoplasms, endoscopic or surgical papillectomy is performed, but intra- and post-operative detailed pathologic examinations are mandatory.

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