Endoscopic ultrasonography (EUS) is useful for detection of lesions, differential diagnosis, tumor staging and therapeutic procedures in patients with biliary diseases. The tumor staging for biliary cancer can be determined by the correlation between the tumor echo and the structure of the wall or the surrounding organs. As for ampullary neoplasm, EUS is useful for diagnosis of pancreatic parenchymal invasion by the tumor and tumor infiltration into the bile/pancreatic duct.
The transpapillary endoscopic approach is sometimes difficult due to ampullary invasion by the tumor or difficult biliary cannulation. Numerous studies regarding EUS-guided biliary drainage in patients with malignant biliary stricture have been reported. Moreover, EUS-guided gallbladder drainage has also been reported to be useful for the treatment of acute cholecystitis. Standardization of these techniques and development of dedicated devices optimal for EUS-guided procedures are necessary.

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We report our experience with 3 cases of adenosquamous carcinoma of the extrahepatic bile duct with characteristic of the imaging findings, as well as clinical and pathological findings. All 3 patients were men, whose average age was 72.3 years. The chief complaints were jaundice in 2 patients and abdominal pain in 1 patient. Macroscopic examination of the resected specimen revealed an expansive nodular tumor in 2 patients and an infiltrating nodular tumor in 1 patient. The tumor was 75%, 65%, and 85% part squamous cell carcinoma, respectively, being a mixture of tubular adenocarcinoma and squamous cell carcinoma in all cases. On histological examination, the squamous component was predominant at sites of infiltration. One patient had lymph node metastasis, which was caused by poorly differentiated adenocarcinoma. Imaging of the bile duct revealed focal stricture, with unilateral compression in all patients. EUS and IDUS revealed a mass growing outward from the bile duct with increased internal echo in all patients. Bile duct stricture with unilateral compression, expansive outward growth, and increased internal echo seem to be characteristic of adenosquamous carcinoma of the bile duct.

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We conducted a nation-wide survey to elucidate the characteristics of PSC as well as IgG4-SC without apparent pancreatic involvement diagnosed after 2005, by sending questionnaires. One-hundred and ninty-seven patients with PSC and 43 patients with IgG4-SC were identified. Compared to PSC, IgG4-SC was male-dominant and developed in older patients. There were two peaks in the age distribution of PSC patients, as demonstrated in the previous nation-wide surveys. The main location of involved bile ducts was both intra and extrahepatic in PSC and intrahepatic in IgG4-SC. Inflammatory bowel diseases was noticed in 68/197 (34%) of PSC, relatively low as shown in the previous surveys. Three-year survival rate were 85.0% in PSC and 90.0% in IgG4-SC, suggesting better prognosis of IgG4-SC.

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Intraductal papillary neoplasm of the liver (IPNB) is characterized by dilated intrahepatic bile ducts filled with a noninvasive papillary or villous biliary neoplasm covering delicate fibrovascular stalks. Dilated bile ducts are fusiform or cystic. One-third of IPNB secrete mucin in the duct lumen (mucin secreting IPNB). IPNB is classified into adenoma, carcinoma-in-situ and invasive carcinoma according to differentiation or into pancreaticobiliary type, intestinal type, gastric type, and oncocytic type according to cytological types, which are considered to be associated with prognosis. IPNB develops predominantly in the left lobe and can be diagnosed using US, CT and MRI. Superficial spread along the bile duct epithelium can be evaluated by choledochoscopy. Although curative resection contributes to good prognosis, re-evaluation of diagnostic criteria, treatment strategy and prognosis is mandatory.

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Small cell carcinoma of the gallbladder is a rare disease process with approximately 15 resected cases in Japanese literatures. The prognosis of the disease is quite poor. A multidisciplinary approach to treatment with surgical resection and adjuvant chemotherapy may be the current standard of care, although neither standard procedure of resection nor standard regimen of chemotherapy had been established. We herein report a case of small cell carcinoma of the gallbladder. A 80-year-old woman with abdominal pain was admitted to our hospital in December 2011. She underwent abdominal CT and US that showed gallbladder cancer with nodal involvement. The liver bed and common bile duct resection was performed in February 2012. The postoperative pathological findings revealed small cell carcinoma, 3.5×3.5×1.0 cm, pT3 (se, INFγ, ly3, v3, pn1, pHinf0, pHM0, pDM0, pN (+), P0, H0, M (-). The immunohistochemistry showed that positive for Synaptophysin, negative for Chromogranin A, and positive for CD56. She has been followed in our satellite clinic as of 2 months after the resection. The collection of the cases with small cell carcinoma of the gallbladder should be essential to elucidate the nature of the disease.

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A 41-year-old woman was visited for examination of the gallbladder. Ultrasonography revealed nodular lesions of 10 mm in the gallbladder fundus and body, with the body lesions showing a higher signal intensity than the fundus lesions on high b-value diffusion-weighted MRI (DWI). We performed open cholecystectomy, suspecting of gallbladder cancer. Pancreaticobiliary maljunction (PBM) was diagnosed by intraoperative cholangiography. In the resected specimen, well-differentiated adenocarcinoma had spread to the entire mucosa, within which the nodular lesions in the fundus consisted of poorly differentiated adenocarcinoma, while the nodular lesions in the body consisted of endocrine cell carcinoma. We subsequently diagnosed adenoendocrine cell carcinoma of the gallbladder complicated by PBM. This case showed a variety of histological types with differing degree of differentiation. Differences in tissue were moreover reflected in signal intensity.

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A case of cholecystoduodenal fistula and duodenal ileus diagnosed by magnetic resonance imaging (MRI) preoperatively is reported herein. A 91-year-old man with vomit was referred to our institute. Abdominal computed tomography revealed pneumobilia and distended proximal duodenum with an impacted gallstone. MRI showed a cholecystoduodenal fistula as well as an intense inflammatory response around the hepatoduodenal ligament. The gallstone was simply removed through an enterotomy without cholecystectomy because of patient's condition and severe adhesion to the surrounded organs. Diagnosis of enterobiliary fistula by MRI is easy and helpful for decision make for a safer surgical approach.

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A 76-year-old male with a complaint of obstructive jaundice was admitted to our hospital. CT and MRI demonstrated hilar mass and dilated intrahepatic bile duct. PET/CT image showed abnormal FDG uptake in the same lesion. ERCP demonstrated the hilar biliary stenosis and IDUS showed a hilar mass with invasion into surrounding tissue. Brushing cytology revealed class V. We diagnosed this case as a hilar cholangiocarcinoma preoperatively and underwent right hepatectomy and resection of extrahepatic bile duct. After histopathological examination, marked infiltration of IgG4-positive plasma cells and fibrosis were observed around the bile duct. Finally, this case was diagnosed as an Immunoglobulin G4-related sclerosing cholangitis.

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Endocrine carcinomas of the gallbladder are reported to show a poor prognosis; thus, early diagnosis plus effective therapy are required. We reported two cases of the neoplasms, which showed no elevation of carcinoembryonic antigen or carbohydrate antigen 19-9, and same image findings: ultrasound demonstrated a homogeneous hypoechoic oval gallbladder tumor beneath a granular innermost hyperechoic layer and endoscopic retrograde cholangiography revealed granular contour of the gallbladder tumor. After undergoing cholecystectomy with liver bed fatty tissue extirpation and lymph node dissection, they received postoperative adjuvant chemotherapy. One case visited another hospital because of lumbar and back pain 13 years after surgery and died of liver metastases with carcinomatous peritonitis due to pancreas body cancer 5 months later. The other case died of lymph node metastases 2 years and 8 months after surgery. Ultrasound and pathological correlation in the cases demonstrated that a granular innermost hyperechoic layer and a hypoechoic oval tumor represented a papillary adenocarcinoma confined to the mucosa and an endocrine carcinoma invading the subserosa, respectively. We thereby stress the significance of the ultrasound finding "submucosal hypoechoic tumor with granular mucosal surfaces" for the diagnosis of endocrine carcinoma.

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While a 53-year-old female was being treated for a gallbladder polyp and pancreatic cystic disease. ERCP revealed a mass suggesting a polyp in the gallbladder. There was neither pancreatic duct abnormality nor pancreaticobiliary maljunction. Cytological examination of bile and pancreatic juice showed trophozoites of Giardia lambia. After treatment with metronidazole, surgery was performed. Laparoscopic cholecystectomy was performed. Pathological examination showed foamy cell aggregation in the lamina propria, and the diagnosis of a cholesterol polyp was made. No Giardia lambia was observed on a whole mount section. She had no history of traveling overseas, homosexuality, or opportunistic infection, and a diagnosis of asymptomatic lambliasis was made. There are also reports of lambliasis and was confirmed to about 0.5% of the subjects colonoscopy also in literature, was considered if there is a relatively common disease. Although this disease is a rare disease itself, it was suggested that the possibility of chronic infection of this disease has been overlooked as a cause of biliary tract disease.

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A 75-year-old man was admitted complaining of abdominal pain and fullness. Ultrasonography revealed the gallbladder with debris, stones and isoechoic tumor measuring 30 mm sited near the gallbladder neck. The tumor was mildly enhanced by CT. By MRI, it demonstrated mildly low intensity on T1-weighted image, mildly high intensity on T2-weighted and high intensity on diffusion-weighted images. Based on these findings, the patient underwent cholecystectomy, resection of the extrahepatic bile duct and regional lymph node dissection (D2) with the preoperative diagnosis of cystic duct carcinoma or carcinoma arising from the gallbladder neck. The tumor was squamous cell carcinoma of the cystic duct. Though postoperative external radiation was applied to surgical margin, the patient died 7 months after the surgery. To our knowledge, this is the first report of primary squamous cell carcinoma of the cystic duct in the English or Japanese literature.

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A 71-old woman, presented with jaundice, was detected dilatation of intra and extra hepatic bile duct on ultrasonography. CT revealed a tumor with contrast enhancement in the lower part of the bile duct. The finding of duodenoscope depicted swollen duodenal papilla with ulcerated mucosa and mucosal convergence. The result of biopsy specimen showed adenocarcinoma. Only suprapapillary incision enabled the deep cannulation to the bile duct. The feature of ERC showed cystic dilatation of the lower part of the bile duct without communication with pancreatic duct. EUS and IDUS revealed also supported the findings. The diagnosis carcinoma of the ampulla of Vater, developed from Ab, associated with choledochocele was made. This choledochocele had no common channel without dilated common bile duct. However, probe operation was undergone due to the multiple micro liver metastaisis.

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This report provides an overview of the use of radiotherapy for biliary tract cancer. Radiotherapy for biliary tract cancer is mainly categorized as adjuvant therapy for resected cases or as therapy intended to achieve local control or symptom palliation for unresectable cases. Due to the lack of randomized controlled trials, the role of radiotherapy in the treatment of biliary tract cancer has not been fully established. However, based on the data from many retrospective studies, chemoradiotherapy can be considered one of the standard treatment options for patients with a positive surgical margin or unresectable biliary tract cancer under the present circumstances. There are many issues regarding radiotherapy for biliary tract cancer that should be examined, such as the role of brachytherapy and the application of new technologies such as intensity-modulated radiation therapy, and thus well-designed clinical trials are needed in this field.

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