A total of 165 lesions of intraductal papillary neoplasm of bile duct (IPNB) were pathologically examined with an emphasis on four subtypes (intestinal, gastric, pancreatobiliary and oncocytic subtypes). The intestinal subtype was common followed by gastric, pancreatobiliary and oncocytic subtypes. These four subtypes presented unique histopathological features and pathological, biological features. They were dicussed with a reference to type 1 and 2 subclassification proposed by Japan-Korea experts biliary pathologists. IPNBs were recommended to be evaluated with a reference to four subtypes and type 1 and 2 subclassification.

Background: The strategy for choledocholithiasis in surgically altered anatomy (SAA) has changed over time. This study aimed to clarify the current status of endoscopic treatment.

Patients and Methods: We retrospectively reviewed 491 patients with choledocholithiasis who received endoscopic treatment from 2008 to 2019. The patients with SAA were divided into early group (2008-2013, n=17) and late group (2014-2018, n=26). The patients with normal anatomy were categorized as normal group (n=448). The treatment outcome was evaluated.

Results: The usage rates of double-balloon endoscope (DBE) and salvage therapy in the late group were increased compared with that in the early group. The stone removal rates in the early, late and normal groups were 70.6%, 100% and 98.7%, respectively. The outcome in the late group was superior to the early group and equal to the normal group.

Conclusions: DBE and appropriate salvage therapy increase stone removal rate in patients with SAA, which can be comparable to those in patients with normal anatomy.

Routine preoperative biliary drainage (PBD) for malignant distal biliary obstruction is not recommended in cases undergoing upfront surgery due to the lack of advantage in PBD. However, PBD is still performed in clinical practice because early surgery is not always possible and biliary drainage is necessary in cases with concomitant cholangitis or in cases undergoing neoadjuvant chemo (radiation) therapy (NAC). While a self-expandable metallic stent (SEMS) appears to provide better stent patency than a plastic stent (PS), there are still some concerns on the use of SEMS as PBD because of possible inflammation along the bile duct. Few data are available on SEMS vs. PS in cases undergoing upfront surgery and NAC in Japan, and further investigations are necessary.

The concept "sclerosing cholangitis" includes various diseases with different clinical courses and therapeutic indications. PSC reveals inflammations and fibrosis in all the levels of the bile ducts, but liver biopsy of the lesion tissues is difficult due to a non-uniform distribution of the lesion. However, a liver biopsy is often necessary for diagnosing PSC in children. Active inflammation containing granulation tissues rich in inflammatory cells may be present in the large bile duct, and can be obtained by a bile duct biopsy. IgG4-related sclerosing cholangitis occurs in the large bile ducts, but aquisition of the lesional tissues by a bile duct biopsy is difficult because of an intramural distribution of lymphoplasmacytic infiltration and fibrosis. When the lesions occur in the intrahepatic bile ducts, inflammatory cell infiltration that extends along the portal tracts can be obtained by a liver biopsy. Among various diseases that show secondary sclerosing cholangitis (SSC), eosinophilic cholangitis, immune-related adverse events caused by immune checkpoint inhibitors, and SSC in critically ill patients are mentioned briefly in this review.

An 82-year-old man was referred to the hospital because of weight loss and anemia. After various imaging tests, bile duct cancer was suspected. Endoscopic sphincterotomy (EST) was performed after definitive diagnosis using endoscopic cholangiography (ERC); hemostasis was performed because of bleeding from the incision. The next day, because of the progression of anemia, an upper gastrointestinal endoscopy was performed, revealing active bleeding at the same site. Hemostasis was obtained by applying pressure on the fully-covered metal stent. Two days later, a clot adhered to the same site; there was active bleeding upon removing the clot. Because of intractable bleeding, complete hemostasis was achieved using a combination of absorbable tissue reinforcement and fibrin adhesive materials at the site of bleeding. This method has been reported to be relatively safe for controlling bleeding after endoscopic submucosal dissection and rectal ulcer bleeding and is also considered to be useful as a hemostatic method for intractable hemorrhage after EST.

A 72-year-old man was diagnosed with advanced gallbladder cancer with liver (S3, S4) and lymph node metastases. After administration of gemcitabine plus S-1 (GS) chemotherapy for 11 months, abdominal computed tomography indicated shrinkage of the main tumor, S3 metastasis, and lymph nodes and disappearance of S4 metastasis. Therefore, conversion surgery was performed. Pathological findings revealed gallbladder cancer with residual lymph node and liver S3 and S4 metastases. Ultimately, the patient was able to survive for 39 months after diagnosis. Conversion surgery might be an effective treatment option for unresectable gallbladder cancer with liver metastases.

A 75-year-old man underwent a laparoscopic low anterior resection for rectal cancer. The postoperative histopathological diagnosis was T3N1bM0, Stage IIIb. Twenty-eight months after surgery, a single liver metastasis was identified at the Segment 5 and a partial hepatectomy was performed. Histopathologically, the tumor was accompanied by macroscopic biliary invasion. After the hepatectomy, bile spillage from the cut surface of the liver developed into a biloma that required percutaneous drainage. A follow-up CT at 6 months after the hepatectomy showed the biloma decreased in size. However, 11 months after hepatectomy, the tumor had grown and became a solid mass. Therefore, a repeat hepatectomy was planned 13 months after the first hepatectomy. Since intraoperative findings revealed that the tumor had infiltrated the diaphragm, the surgical procedure became a right lobectomy with diaphragm resection. Histopathological diagnosis revealed that most of the tumor was located outside of the liver capsule. Based on these findings and the clinical course, image findings over time, we diagnosed the patient with a local recurrence developed from biloma after surgery for liver metastasis. Two years after the repeat hepatectomy, the patient is alive without recurrence.

We herein present a rare case of a carcinoma that originated from the peribiliary gland within the ampulla of Vater. A 77-year-old male with epigastralgia was referred to our hospital for further examination and possible surgery. Under a strong suspicion of a carcinoma of the ampulla of Vater, pancreatoduodenectomy was performed. Microscopically, the tumor was a moderately to poorly differentiated adenocarcinoma. The tumor was distributed primarily in the submucosal layer of the duodenum, and not in the mucosa of either the bile duct or pancreatic duct. Immunohistochemical analysis of the tumor showed a pancreatobiliary phenotype, indicating that the tumor was not likely derived from the duodenal mucosa. Based on these findings, a carcinoma of the ampulla of Vater arising from the peribiliary gland was diagnosed. This reported case appears to be a very rare case of a carcinoma of the ampulla of Vater from the viewpoint of tumor development and extension.

A 74-year-old man who underwent Sigmoidectomy due to Sigmoid colon cancer in October 2018 was introduced to our hospital because of left intrahepatic bile duct dilation. Enhanced CT scan revealed the intrahepatic bile duct dilation and the enhanced mass in junction of B4. MRCP revealed the obstruction in junction of B4. Peroral cholangioscopy (POCS) showed the papillary mass in junction of B4 to left hepatic duct and the biopsy revealed class V. The operation was performed with diagnosis of intrahepatic cholangiocarcinoma on January 2019. We recognized the small white mass on the liver surface during the surgery and judged as liver metastasis of sigmoid colon cancer. All lesions were limited in the liver left lobe and the intraoperative frozen section revealed that the resection stump of left bile duct was cancer negative, so the left lobectomy was performed. Histopathological findings were adenocarcinoma and Cytokeratin (CK) 7 (-), CK20 (+), CDX2 (+) in the both lesion of bile duct and liver, so the origin was colorectal cancer. The diagnosis was sigmoid colon cancer with synchronous bile duct and liver metastasis.

A 16-year-old-male was introduced to our hospital for the purpose of examining the etiology of pancreatitis. Abdominal and endscopic ultrasonography showed gallbladder wall thickness. Endoscopic retrograde cholangiopancreatography revealed pancreaticobiliary maljunction (PBM) without dilation. Biliary amylase level was high (302,300U/l). Gallstones, pancreatic tumors and autoimmune diseases were negative. Combined bile duct resection with cholecystectomy was performed for this PBM case, because pancreatitis was due to PBM. Histopathological examination of the resected specimens revealed no malignancy. The postoperative complications and recurrence of pancreatitis have not occurred. Combined bile duct resection with cholecystectomy may be effective surgical treatment for this PBM case.

A 60-year-old male was suffered from right hypochondrial pain. He was admitted to our hospital because he suspected gallbladder abnormality by ultrasonography. Laboratory data including tumor markers was normal. US showed cholecystolithiasis. CT and MRCP showed septations in the entire area of the gallbladder. He underwent laparoscopic cholecystectomy. In the operative findings, gallbladder was atrophic and elastic hard due to chronic inflammation. Pathological findings revealed that the inflammatory granuloma was formed in the stroma of the gallbladder wall, and the abscess was also formed. The diagnosis was multiseptate gallbladder with xanthogranulomatous cholecystitis.

Multisepatate gallbladder is a rare congenital disease and there hardly ever has been reports. In addition, this case was complicated because xanthogranulomatous cholecystitis and gallbladder stone were involved. So far, this is the first case report of multisepatate gallbladder with xanthogranulomatous cholecystitis and gallbladder stone. Therefore, herein we report with updated literatures.

Lanreotide is a long acting somatostatin analogue and gallstones are reported in over 5% as a side effect. But there are no previous treated reports of patients for biliary tract stones during lanreotide therapy. We herein report a treatment case of gallbladder and common bile duct stones during lanreotide therapy. A 74-year-old female was diagnosed with a pituitary adenoma and acromegaly in 1985. After two times operations and stereotactic radioterapy, octreotide was started in 2000, and lanreotide given since 2014. In 2018, abdominal pain began after colonoscopy and a splenic hematoma suspected on abdominal CT scan and admitted urgently. Laboratory check pointed out liver dysfunction and further evaluations revealed gallbladder and common bile duct stones. After removing the common bile duct stone endoscopically, laparoscopic cholecystectomy was performed. Componential analysis of stone revealed that the stones were calcium bilirubinate gallstones including over 98% calcium bilirubinate.

A 60-year old man was referred to our hospital due to necrotizing cholecystitis and laparoscopic cholecystectomy was performed. A necrotized gallbladder was recognized and necrotized cystic duct was ligated twice by absorbable monofilament suture and dissected. Immediately after the operation, bile leakage was detected in drainage tube. The leakage of a contrast medium was found nearby cystic duct stump by ERC but the leakage point was unclear. We considered the reduction of common bile duct pressure with stable patency of biliary stent. Therefore, the patient received covered-metal stent and the bile leakage disappeared on the 11 days after placement of stent. Removal of stent was performed on the 4 month after operation. The remnant of cystic duct was contrasted but leakage was not detected. In the point of quality of life, covered-metal stent was acceptable and efficient as a treatment option of bile leakage after laparoscopic cholecystectomy.

We report a case of a 50-year-old female with double cancer that developed 49 years after choledochoduodenostomy for a congenital biliary dilatation (CBD) at 3 months of age. She presented with epigastralgia and was diagnosed as having distal cholangiocarcinoma near the anastomosis of the cystoduodenostomy due to upper gastrointestinal endoscopy findings. After she was admitted to our department, we diagnosed her as having double cholangiocarcinoma of the perihilar and distal bile duct and performed subtotal stomach-preserving pancreaticoduodenectomy including perihilar bile duct resection. Well-differentiated adenocarcinoma in the perihilar bile duct and poorly-differentiated adenocarcinoma in the distal bile duct were observed in the excised specimen. No gross or pathological continuity was observed between the lesions. The patient has remained recurrence-free as of 1 year and 2 months postoperatively. We should recognize that patients who have undergone choledochoduodenostomy for CBD may develop cancer of the residual biliary system and periodically monitor them considering the possibility of double cancer, as in the present patient.

Although various kinds of drainage methods to unresectable malignant biliary obstruction has been reported, consensus regarding stenting strategy has not been established. Currently, the symptomatic patients, such as jaundice fever, usually undergo endoscopic transpapillary biliary drainage. In case of distal obstruction, metallic stents are often used, however, there are various options in case of hilar obstruction. Recently, prognosis of the patient with malignant hilar biliary obstruction has been prolonged with the advance in anti-tumor therapy, and we should choose the stenting method considering the feasibility of re-intervention in addition to the initial stent patency on the choice of stenting method. In recent years, EUS-guided biliary drainage (EUS-BD), in which a stent is deploy by puncturing the bile ducts using a linear echoendoscope, has attracted much attention as an alternative drainage route. The endoscopists should perform appropriate procedure according to each biliary stenting after the confirmation of the shape of biliary stricture with imaging study.