Cases reported "Cholangiocarcinoma"

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1/22. Biliary papillary hyperplasia with clonorchiasis resembling cholangiocarcinoma.

    infection by the liver fluke clonorchis sinensis is very common in the far east. It causes low grade inflammatory changes and proliferation in the biliary tree. Initially there is desquamation of the biliary epithelium, followed by hyperplasia and adenomatous proliferation. Cholangiocarcinomas are potential long term complications. We present a case of biliary papillary hyperplasia with clonorchiasis resembling cholangiocarcinoma in a 69-yr-old Korean man. Early recognition of biliary hyperplasia and treatment of clonorchis sinensis is important to prevent development of cholangiocarcinoma, especially in the far east.
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2/22. Mucobilia in association with a biliary cystadenocarcinoma of the caudate duct: a rare cause of malignant biliary obstruction.

    Mucobilia is a rare condition characterized by the accumulation of abundant mucus within the intra- or extrahepatic biliary tree. A variety of hepatobiliary and pancreatic neoplasms are mucin producing and have been associated with the development of mucobilia including biliary mucinosis, biliary papillomatosis, mucin-producing cholangiocarcinoma (MPCC), or cystic neoplasms of the pancreas or biliary tree (cystadenoma or cystadenocarcinoma). We report the case of 46 year-old male with a biliary cystadenocarcinoma of the caudate lobe which resulted in chronic biliary obstruction and relapsing cholangitis. A review of the literature for both mucobilia and biliary cystadenocarcinoma is provided along with a discussion addressing the clinical presentation, diagnosis, treatment, and prognosis for this rare entity.
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3/22. Fine-needle aspiration cytologic diagnosis of intrahepatic biliary papillomatosis (intraductal papillary tumor): report of three cases and comparative study with cholangiocarcinoma.

    Biliary papillomatosis is a rare tumor of the intrahepatic and extrahepatic biliary tree, and its FNA findings have not been reported. The cytologic features of 3 cases of intrahepatic biliary papillomatosis were studied and compared with 5 cases of cholangiocarcinoma. The distinctive features include: 1) hypercellular smear, 2) very broad and often double-cell layered sheets of ductal columnar epithelium, 3) papillary configuration, 4) preserved honeycomb pattern with even nuclear spacing, and 5) dysplastic but not frankly malignant nuclear features. The constellation of these features is highly characteristic of biliary papillomatosis and helpful in distinguishing it from cholangiocarcinoma and other differential diagnoses. A firm preoperative diagnosis can thus be achieved, allowing better planning in management of this borderline malignant tumor.
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4/22. Recurrent cholangiocarcinoma: negative autopsy results after aggressive management.

    cholangiocarcinoma is one of the most common malignancies of the biliary tree. Most cases are perihilar. cholangiocarcinoma usually has an indolent, slowly progressive course and is associated with a high mortality rate. In this article, we discuss the management of perihilar cholangiocarcinoma by radiation therapy and chemotherapy in a case in which no recurrence was found on autopsy.
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5/22. hepatectomy for cholangiocarcinoma complicated with right umbilical portion: anomalous configuration of the intrahepatic biliary tree.

    The right umbilical portion (right-sided round ligament) has been discussed as an intrahepatic portal venous anomaly associated with "left-sided gallbladder" in several reports. We treated two patients with right umbilical portion (RUP) associated with cholangiocarcinoma. Left hepatectomies were performed, preserving the residual hepatic blood flow and biliary continuity. From our experience in these patients we propose the presence of anomalous configuration of the intrahepatic biliary tree in RUP, because both patients showed medial segmental bile ducts ramified from the right and left hepatic ducts. In general, although the medial segmental bile duct ramified from the left, we surmised that this abnormal bilateral drainage pattern may not be a rare phenomenon in RUP. Special attention may be required to focus on the anatomy of the portal tributaries and biliary ramifications in RUP.
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6/22. Intrahepatic cholangiocarcinoma arising 10 years after the excision of congenital extrahepatic biliary dilation.

    A 52-year-old woman was found to have a liver tumor during treatment for a liver abscess. The tumor was diagnosed as intrahepatic cholangiocarcinoma by closer examinations, including a percutaneous needle biopsy. Ten years previously, she had undergone excision of a choledochal cyst, with reconstruction by Roux-en-Y hepaticojejunostomy, as treatment for Todani's type Ia congenital biliary dilation, which had been confined only to the extrahepatic bile duct. The significant association between congenital biliary dilation and hepatobiliary malignancies is well known. Some patients have been reported to develop biliary cancer long after the excision of the entire extrahepatic bile duct and hepaticoenterostomy. However, in these patients, the development mostly took place in the remnant choledochal cyst, the anastomotic site, or in the dilated intrahepatic bile duct of Todani's type IV-A congenital biliary dilation. The development of intrahepatic cholangiocarcinoma after operation has not been reported previously in a patient with Todani's type I congenital biliary dilation. This case suggests that the entire biliary tree may have a high risk of field cancerization, even in extrahepatic congenital biliary dilation.
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7/22. Diffuse mucosal carcinoma of intrahepatic and extrahepatic bile ducts including gallbladder.

    An autopsy case with a widespread mucosal carcinoma of the biliary tree was reported. A biochemical profile of the bile duct damage was noticed in a woman in her seventies during a gastric examination. Imaging procedures depicted irregular dilatations of intrahepatic bile ducts with a bead-like appearance. Elevated levels of serum alkaline phosphatase and gamma-glutamyltransferase with a negative antimitochondrial antibody persisted. The patient was diagnosed as primary sclerosing cholangitis, she was followed up for 4 years under preservative therapies, and died of anasarca and heart failure. Post-mortem examination showed a diffuse mucosal carcinoma of both intrahepatic and extrahepatic biliary passages including the gallbladder with a minimal invasion and scattered foci of adenoma-like area in part. There was no evidence of gallstones or pre-existing sclerosing cholangitis. The striking features of the tumor were extensive papillary growth, mucus secretion and irregular dilatation of bile ducts. The tumor may bear biological and morphological homology with intraductal papillary mucinous tumor of the pancreas.
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8/22. Early neoplasias of the gallbladder and bile duct: an "unstable" biliary epithelium?

    Benign tumours of the biliary tree are rare. In particular, only anecdotal cases of intraductal villous adenomas have been reported. The polyp-cancer sequence has not been observed in the biliary epithelium, in contrast to the paradigm of colorectal carcinogenesis. This report presents the case of a 64-year-old woman with a past history of cholelithiasis who had two early neoplasias involving the biliary epithelium: an adenocarcinoma in situ of the gallbladder and a common bile duct (CBD) villous adenoma with high-grade dysplasia. The tumours presented 4 years apart. The clinical features and combined radiological, cytological, and surgical modalities leading to the diagnosis of intraductal villous adenoma are presented. The endoscopic ultrasound (EUS) characteristics of villous adenoma of the CBD are described. While the prognosis on both occasions appears excellent following curative resections of both tumours detected at an early stage, it is possible that further neoplasia involving the biliary tree may recur. There are currently no data on optimal surveillance modalities. It may be hypothesized that the gallbladder and biliary epithelium share a similar mechanism for carcinogenesis to that observed in the colonic adenomacarcinoma sequence.
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9/22. hepatectomy for proximal bile duct carcinoma in a patient with situs inversus; a case report.

    situs inversus is a rare condition which mandates a full understanding of all anatomic relationships prior to invasive procedures. A 76-year-old woman with situs inversus presented with fever and rigors. She had previously undergone endoscopic sphincterotomy and lithotomy for choledocholithiasis, and laparoscopic cholecystectomy for cholecystolithiasis. Laboratory examination revealed hyperbilirubinemia and transaminasimia. Percutaneous transhepatic biliary drainage, percutaneous transhepatic cholangioscopy, percutaneous transhepatic portography, percutaneous transhepatic portal embolization, and visceral angiography were performed without complications. She underwent right hepatic lobectomy, caudate lobectomy and extrahepatic bile duct resection for papillary adenocarcinoma of the proximal bile duct. Full investigation of the anatomical relationships between the biliary tree and the vascular system in the hepatic hilus enabled safe hepatectomy in a patient with situs inversus.
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10/22. Advanced cholangiocarcinoma in a patient with stage I primary sclerosing cholangitis.

    A 29-year-old woman presented with jaundice and fever in May 2001. cholangiography showed multiple strictures and beading of the biliary tree, with a large stricture in the common bile duct and marked dilatation of the hilar bile ducts. Typical cholangiography findings and elevated hepatobiliary enzymes suggested primary sclerosing cholangitis (PSC). At the same time, computed tomography detected a 2-cm tumor in the common bile duct, and angiography showed an encasement in the portal vein. Tumor markers, cytology, and biopsy were all negative for cancer. Although laparotomy showed a healthy liver and no lymph node metastasis was found, suggesting early-stage PSC and a low likelihood of accompanying cholangiocarcinoma (CCA) reported so far, the tumor in the resected common bile duct was subsequently diagnosed as CCA. Therefore, pancreatoduodenectomy was performed combined with partial resection of the portal trunk. histology also revealed invasion of the wall of the portal vein by cancer cells. The patient had a recurrence 5 months later and died 12 months after her operation. This is a rare case in which stage I PSC was complicated by advanced CCA.
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