Although surgical resection provides the only chance of cure for perihilar cholangiocarcinoma, it easily invades to around organs such as liver, pancreas, portal vein or hepatic artery by longitudinal and radial spreads. Aggressive resection improves treatment outcome, but it tends to be high invasive surgery with combined resection of around organs. Therefore it is very important to determine the surgical indication or operative procedure by accurate preoperative diagnosis for longitudinal and radial extension.

Multidetector-row CT (MDCT) has high spatial and time resolution. We can make multiplanar reformat imaging or three dimension imaging from volume data of MDCT. Herein I show the delineation and diagnosis for longitudinal and radial extension in perihilar cholangiocarcinoma by MDCT with the contrast between CT imaging and pathological findings.

The use of long-term endoscopic gallbladder stents (EGBS) has recently reported to prevent recurrence of acute cholecystitis in high-risk surgical patients. However, many points require clarification. We retrospectively analyzed patients who underwent EGBS placement and followed up those who underwent long-term stenting (EGBS group; n=30) and those in whom the stent was removed (removal group; n=10). The median event-free survival time was significantly longer in the EGBS group than in the removal group (870 days vs. 166 days; P = 0.043). Recurrence of symptoms was not observed in the EGBS group less than a year and the proportion of recurrence was significantly lower in the EGBS group (7% vs. 50%; P = 0.006). Late complications occurred in six cases (20%) in the EGBS group (five complications occurred after one year with three deaths). Long-term EGBS is considered to be effective in preventing recurrence of cholecystitis. However, late complications, including death, have been observed. It is thus recommended to replace the stent yearly.

Recently, endoscopic ultrasound-guided gallbladder drainage (EUS-GBD) has attracted much attention. However, the procedure is rather unstable. One of the causes was the lack of adhesion between the walls of the gallbladder and the digestive tract. We developed an anchoring device to fix the walls as a pretreatment. The gall bladder and stomach were fixed under EUS in four biologic pigs using the anchoring device. The fixing of stomach and gallbladder walls was performed in 3 pigs, and technical success was achieved in all 3 cases. In the 2 cases that were reared on the 17th and 34th, only 1 case could be fixed, but in 1 case, the anchoring of the gallbladder and the gastric wall was off because the wing of the anchoring device came off the gallbladder. Although there remain issues regarding efficacy and safety, we plan to make improvements and aim for clinical application.

Endoscopic ultrasound/endosonography guided-biliary drainage (EUS-BD) is now covered by the Japanese insurance and the clinical practice guidelines were recently published, but EUS-BD has not been standardized yet. For safe performance of EUS-BD as well as safe introduction of the procedure to non-expert centers, there are some hurdles such as the lack of dedicated devices, needs for technical skills and potential risks of severe adverse events. Since there are few currently available evidences, the clinical practice guidelines were mainly based on expert opinion, not high level evidences, and there still remain many important issues. Establishment of technical terms and classification of procedures are required urgently and many clinical trials are mandatory to define indications, device selection and management of adverse events. Furthermore, consensus are necessary to establish the methods of education and introduction to non-experts and their centers. We should conduct clinical trials to make high quality evidences and hopefully Japanese endoscopists will lead the global situations and contribute to the safe spread this procedure all over the world.

Intraductal papillary neoplasm of the bile duct (IPNB) is considered as a counterpart of intraductal papillary mucinous neoplasm of the pancreas (IPMN), however, IPNB has not been re-evaluated based on the similarity of pathological findings to IPMN, occurrence site, mucin production, and so on. This caused unbalanced case collection, contributing to the differences of diagnosis, treatment and results. In this study, we proposed a new classification; the lesions which pathologically resembled IPMN, frequently occurred at intrahepatic and perihilar bile duct, and showed minimal invasion with good prognosis, might be diagnosed as Type 1, while those which slightly resembled IPMN, frequently occurred at the distal bile duct, did not produce mucin and showed invasion with poor prognosis, were classified as Type 2. There were significant differences in the serum levels of liver enzymes, and total bilirubin, mucin production, occurrence site, grade of invasion and cumulative survival rate between the two types. We have to validate the appropriateness of the classification.

Drug therapy plays a major role in the treatment of advanced biliary tract cancer, but its efficacy and treatment option are limited. Therefore, further development is indispensable for improving the overall prognosis of this cancer. Precision medicine, which examines genetic alterations in cancer and treat it with molecular targeted agents, is a new treatment option which is particularly expected for biliary tract cancer. Recently, several FGFR inhibitors and IDH inhibitors showed good efficacies and tolerability for advanced intrahepatic cholangiocarcinoma. In Japan, a cancer multi-gene panel testing has been approved by public insurance, and it is expected that treatment based on genetic alteration will further develop in the field of biliary tract cancer. However, there are many issues that needed to be resolved in the near future, such as the time until the results of genomic testing are obtained, the way to gain a sufficient volume of sample in inoperable cases, the lack of effective therapeutic agents for genetic alterations, and the cost of genomic testing.

A 78-year-old woman was confirmed the dilatation of the intrahepatic bile duct by abdominal ultrasonography and was referred to our hospital. Contrast-enhanced CT showed a 2cm-sized tumor in the vicinity of the left hepatic duct. ERCP showed a stenosis in the left hepatic duct and the anterior segment, and was negative for cancer by bile cytology and bile duct brushing cytology. Biopsy was performed a cholangioscope (spyglass^®), but the cancer was negative. Although no cancer was diagnosed histopathologically, it was difficult to rule out the cancer due to sampling errors and the possibility of mass-forming intrahepatic cholangiocarcinoma, so we performed left trisectionectomy, caudate lobectomy, and extrahepatic bile duct resection. After surgery, she had bile leakage but was relieved by drainage and discharged on the 42nd hospital day. Pathological examination revealed that the mass has only fibrosis and inflammatory cells without neoplastic tissues. She was diagnosed with a hepatic inflammatory pseudotumor.

A 68-year-old man underwent total laryngopharyngoesophagectomy for hypopharyngeal cancer. Pathological diagnosis showed poorly differentiated squamous cell carcinoma, pT3N2bM0 StageIVA. Two years after surgery, PET-CT showed abnormal uptake in the gallbladder. Contrast enhanced abdominal CT revealed consecutive tumors of 30mm in the fundus and 25mm in the neck of the gallbladder. MRI showed similar findings, thus the gallbladder cancer was suspected. Hepatectomy of segment 4a and 5 was performed. There was no lesion in the mucosa and the histology was similar to that of a previous hypopharyngeal cancer. Final pathological diagnosis was gallbladder metastasis of hypopharyngeal cancer. Gallbladder metastasis of hypopharyngeal cancer are extremely rare. Renal cell carcinoma, malignant melanoma, gastric cancer and breast cancer have been reported as the primary tumor of gallbladder metastasis. Gallbladder metastasis of hypopharyngeal cancer has not been reported so far in Japan as well as in the world.

A 69-year-old man, who had undergone extrahepatic bile duct resection for extrahepatic cholangiocarcinoma (papillary adenocarcinoma, pStage IA) 11 years ago presented with fever. He was admitted and diagnosed as acute cholangitis and liver abscess. After resolving with antibiotic therapy, further evaluation was needed due to an elevated tumor marker. Contrast-enhanced CT images revealed a 35-mm mass in segment IV of the liver, which was involving the left branch of portal vain and middle hepatic vein. He was diagnosed as metachronous intrahepatic cholangiocarcinoma, and underwent left lobectomy and left caudate lobectomy with middle hepatic vein resection. Pathological examination revealed well differentiated adenocarcinoma and no evidence of any vascular infiltration. Final diagnosis was Stage II intrahepatic cholangiocarcinoma, and no recurrence was confirmed under 3 years follow-up. Multiple primary cancers of intrahepatic and extrahepatic cholangiocarcinoma were rare; however, it should be noted that a careful follow-up is needed in patients after curative surgery for cholangiocarcinoma, especially in patients with newly onset cholangitis, for early diagnosis of recurrent or metachronous cholangiocarcinoma.

An 81-year old woman underwent laparoscopic left hemi colectomy for transverse and descending colon cancer. In pathological examination, transverse colon lesion was diagnosed as tub2, pMP, ly0, v0, PM0, DM0, pN0, pStageI and descending colon lesion was pap, pMP, ly0, v0, PM0, DM0, pN1, pStageIIIA. In five years later after the operation, CT examination revealed a mass 1cm in size at liver S8. In the dorsal branch of B8, tumor thrombus was detected continuous from the mass. We diagnosed as metastatic liver cancer with bile duct tumor thrombus and performed partial liver S8 resection. In pathological examination, the tumor originated from the bile duct epithelium replaced by atypical cells proliferating into the bile duct. However, the mass did not invaded into liver parenchyma. The primary cholangiocarcinoma of intra biliary growth type and metastatic liver cancer with growth into the bile duct were identified as differential diagnosis. From the immunohistological findings: CK7 was negative, CK20 was negative and CDX-2 was positive, we diagnosed as colorectal biliary metastasis. We report this rare case of biliary metastasis from colorectal cancer that metastasized to the bile duct epithelium and grew into the bile duct without invasion of liver parenchyma.

A 65 year-old female was suspected of having anastomotic stenosis of choledochojejunostomy and cholangitis after subtotal stomach-preserving pancreaticoduodenectomy. Although double-balloon endoscopy was performed, the anastomosis could not be identified. Therefore percutaneous transhepatic biliary drainage was attempted for the purpose of performing the rendez-vous technique, but the guidewire could not pass the stenosis. Double-balloon endoscopy was repeated, and after the endoscope reached the anastomosis, it was replaced with a forward-viewing echoendoscope using a 0.052 inch hard guidewire for the shaft. Endoscopic ultrasound-guided biliary drainage was then performed by using the forward-viewing echoendoscope to directly puncture the common bile duct from the anastomosis. After the balloon dilation, two 7-Fr plastic stents were placed. The procedures were successfully completed without any complications. The stents were removed 3 months later. In summary, we report a new salvage technique for severe anastomotic stenosis of choledochojejunostomy after failed double-balloon endoscopic retrograde cholangiography.

The case was a 56-year-old woman. She had anorexia two weeks ago, and then noticed urinary yellow staining and white stool. Abdominal contrast-enhanced CT showed a 15-mm mass in the pancreas head, causing bile duct obstruction. An 83-mm uterus tumor suspected to be malignant and multiple lung and bone metastases were also found. ERCP was performed, a severe stenosis was observed in the distal bile duct. IDUS showed a low echo mass in the pancreatic parenchyma around the stenosis. EUS-FNA was performed for the pancreatic mass, and dense proliferation of spindle cells with atypical nuclei was observed. Immunostaining revealed leiomyosarcoma with SMA (+) desmin (+). The patient was diagnosed with pancreatic metastasis of uterine leiomyosarcoma, and chemotherapy was introduced after biliary stenting. Uterine leiomyosarcoma with obstructive jaundice is rare and is reported with literature review.

A 71-year-old woman came to our hospital with abdominal pain. We performed cholecystectomy with a preoperative diagnosis of cholecystitis. A firm nodule at the confluence of cystic duct (CD) was not a stone but a mass. With an intraoperative pathological diagnosis of poorly differentiated adenocarcinoma, extrahepatic bile duct resection with lymphadenectomy was performed. A 73-year-old man, her 14-years younger brother, presented to our hospital with abdominal pain. CT revealed the enhanced tumor spreading from the CD to common bile duct. Pancreaticoduodenectomy was performed. Final pathological diagnosis was papillary adenocarcinoma of the CD. They had no other family history of malignancy and never worked at printing factories. Biliary tract carcinoma in siblings is rare. No specific risk factors of familial neoplastic diseases could not be identified in this particular family.

A 69-year old man visited a nearby hospital presenting with jaundice, epigastric pain, and fever. The delated two intrapancreatic bile ducts were detected, opening to the duodenal papilla. One of the double common bile ducts was packed with papillary tumor. The papillary orifice was dilated because of excessive production of mucin. We performed pancreatoduodenectomy. Histologically, columnar tumor cells with various degrees of atypia showed papillary growth within the dilated duct. The also tumor involved the main pancreatic duct. A final diagnosis of IPNB concomitant with double common bile duct was established. There is no report of patient with IPNB concomitant with double common bile duct. For the treatment of the double common bile duct, it is important to adequately classify the types of double common bile ducts and to know the co-morbidity of this rare variants, preoperatively.

A 71-year-old man was referred to our hospital, suspected gallbladder cancer. Contrast-enhanced computed tomography revealed a tumor with contrast effect from the gallbladder body to neck, and liver bed infiltration was also suspected. The extent of cancer was suspected up to the cystic duct, and there was no distant metastasis. Subsequently, S4a+S5 hepatic segmentectomy, extrahepatic bile duct resection, and regional lymphadenectomy were performed for the diagnosis of gallbladder cancer. Simultaneous liver metastasis measuring 5mm in diameter was found in the resected side of liver. The final pathological diagnosis was StageIVB. He had survived recurrence-free for 3 years and 3 months without adjuvant chemotherapy following surgery. In this case, long-term patient survival was achieved by performing S4a+S5 hepatic segmentectomy. In this case, a tumor thrombosis was formed in the portal vein near the gallbladder cancer, suggesting liver metastasis was formed via cystic vein.

We herein report a case of pancreatoduodenectomy (PD) for carcinoma of the bile duct with a replaced common hepatic artery (CHA) arisen from the hepatomesenteric trunk running through the pancreatic parenchyma. A 76-year-old man was diagnosed as carcinoma of the bile duct in clinical stage IIA.

CT showed the replaced CHA coursing through the pancreatic head parenchyma. Since the location of the tumor was distant from the CHA, we determined that the conservation of the CHA could afford to perform a radical operation. SSPPD was performed preserving the CHA after the pancreas parenchyma was transected. There were abundant fragile vessels around CHA. The operation time was 483min, and the intraoperative blood loss was 1386g. Pathologically, the exfoliation margin was negative. The anatomy that the replaced CHA is running through the pancreatic parenchyma is very rare. To perform safe and radical PD, preoperative precise investigation of the anatomy and simulation of the operation are necessary.

A 78-year-old male who had a liver cystic lesion with 60mm diameters on liver S1 was admitted to our hospital for further examination. At the initial visit, he had no chief complaint, and we observed it as a simple liver cyst for eighteen months. However, it has gradually increased and the solid components with abnormal accumulation on FDG-PET have appeared in cystic lesion. We performed left hepatic trisegmentectomy and extrahepatic bile duct resection based on the diagnosis of hepatic mucinous cystadenocarcinoma. Histopathological examination revealed no obvious ovarian-like stroma. It diagnosed as IPNB with an associated invasive carcinoma. We report a rare case of IPNB which showed the morphological changes during follow up.

A 64-year-old man was pointed out a 10mm sized minor papilla tumor by esophagogastroduodenoscopy of medical examination. With endoscopic ultrasound-guided fine needle aspiration, the diagnosis was not confirmed, so endoscopic minor papilla resection was performed. Histopathology diagnosed neuroendocrine tumor (NET) G1 with insulinoma-associated 1 (+), mitotic index 0 / 10HPF, Ki-67 Labeling Index < 1 %, and resection margin was positive and infiltrated to muscularis propria. Therefore, subtotal stomach-preserving pancreaticoduodenectomy was performed and pathologically surgical margin was negative, lymph node metastasis at No.6,14,7b were observed. A minor papilla NET probably causes lymph node metastasis even if the tumor is less than 10mm, so regardless of the tumor size, pancreaticoduodenectomy and standard lymph node dissection is appropriate treatment.

A 69-year-old woman was diagnosed as unresectable locally advanced gallbladder cancer (LAGBC) with invasion to the right hepatic artery and portal vein. She received chemotherapy with gemcitabine and cisplatin. After 9 courses, the size of the tumor decreased and the metastasis was not identified. We planned conversion surgery. On laparotomy, the right hepatic artery was detached from the tumor. Therefore, we performed extended cholecystectomy, extrahepatic bile duct resection and regional lymphadenectomy. Pathological investigation confirmed poorly differentiated tubular adenocarcinoma, pT2 (SS) N0M0, Stage II, and R0 resection margins. The patient received no adjuvant therapy. 17 months after the surgery, she is alive without recurrence. The consensus of conversion surgery for unresectable LAGBC has not been determined. Further studies are required to establish treatment strategy of conversion surgery for unresectable LAGBC.

A 29-year-old male was referred for further examination of epigastric pain. Abdominal CT showed a neoplastic lesion in the hilar bile duct. MRCP showed pancreaticobiliary maljunction (PBM) without dilatation of common bile duct. An extended left hepatectomy with extrahepatic bile duct resection was performed with the diagnosis of hilar cholangiocarcinoma (Bismuth II) associated with non-dilated PBM. Histopathologically, biliary intraepithelial neoplasia (BilIN) lesion was observed in the biliary epithelium continuous to the invasive carcinoma. BilIN spread extensively to the duodenum side of the tumor. High-grade atypical cells (High-grade BilIN) were observed nearby the tumor, suggestively representing carcinogenesis process. This was a rare case of cholangiocarcinoma developed in non-dilated PBM at a young age of less than 30 years.

Hepatectomy for biliary tract cancer is well-known as a high-risk surgical procedure potentially yielding a high morbidity and mortality. Preoperative biliary drainage (PBD) has been routinely used in Japan, which totally conflicts to the indication in the West. However, recent studies demonstrated that PBD reduced the incidence of liver failure and mortality after right or more extended hepatectomy. Percutaneous transhepatic biliary drainage (PTBD) increased the incidences of catheter tract seeding and disseminated disease, thereby reducing postsurgical survival. This observation revises a PBD-strategy from percutaneous approach to endoscopic one, particularly in Japan. In addition, because preoperative cholangitis is highly associated with postoperative mortality, PBD should be chosen on the basis of the potential risk of procedure-related cholangitis. The first-line PBD is endoscopic naso-biliary drainage in Japan; endoscopic biliary stent or percutaneous biliary drainage in the overseas. Thus, the strategy of PBD still differ among regions or hospitals due to lack in scientific evidence.