Cases reported "Cholangiocarcinoma"

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1/6. Synchronous carcinoma of the gallbladder in a patient with intrahepatic bile duct carcinoma.

    An 83-year-old woman, diagnosed as having cholelithiasis, was admitted to the Department of Surgery, Nippon Medical School, with right hypochondrial pain. ultrasonography and computed tomography revealed a mass in the gallbladder fundus and a hypovascular tumor in the anterior segment of the liver. magnetic resonance imaging showed stenosis of the intrahepatic bile duct and dilatation of its proximal portion. She was diagnosed as having intrahepatic bile duct carcinoma combined with gallbladder carcinoma. At laparotomy, there was evidence of multiple peritoneal metastases and intraoperative histological examination of the gallbladder tumor revealed adenocarcinoma. Accordingly, only cholecystectomy and needle biopsy of the liver tumor was performed. Histological examination of the gallbladder revealed papillary adenocarcinoma invading the muscularis propria with medullary growth or intermediate stroma. There was no microvessel invasion, no perineural invasion and no lymph node involvement. On the other hand, the liver tumor was a cholangiocarcinoma with a well-differentiated tubular pattern. Therefore, this was a rare case of synchronous carcinoma of the gallbladder associated with intrahepatic bile duct carcinoma.
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2/6. Resection of a hilar cholangiocarcinoma in a patient with absent portal bifurcation.

    Absence of the bifurcation of the portal vein is an extremely rare anomaly and the misled ligation of the portal vein would result in the lethal hepatic failure. In this paper, a resected case of hilar cholangiocarcinoma with this anomalous portal venous system is first presented. Preoperative percutaneous transhepatic portography disclosed an absence of portal bifurcation and a transversely running portal vessel from the right anterior segment to the left lateral segment. The patient underwent left hepatic lobectomy with caudate lobectomy, in which the portal trunk was successfully preserved.
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3/6. Combined hepatocellular-cholangiocarcinoma presented with massive pulmonary embolism.

    A 30-year-old HBsAg-positive woman was admitted to the hospital because of 6 days of progressive shortness of breath. She was in severe respiratory distress with circulatory collapse. She had an enlarged liver but no stigmata of chronic liver disease or signs of cirrhosis. She had rapidly developed respiratory arrest and was transferred to intensive care unit. heart ultrasonography and Doppler scan showed right heart straining and high pulmonary artery pressure. Despite cardiovascular and respiratory support she died a few hours after admission. autopsy revealed combined hepatocellular-cholangiocarcinoma infiltrating the entire liver, metastatic invasion of lung blood vessels and absence of right ventricular hypertrophy. The incidence of hepatocellular-cholangiocarcinoma, a variant of hepatocellular carcinoma, is roughly 2-3% and the presenting symptoms are abdominal pain, weight loss, jaundice, fever or decompensation of liver disease. Associated HBsAg positivity and cirrhosis are reported in 20-30% and 60% of patients, respectively. Metastases to lungs are relatively frequent but this is the first report of hepatocellular-cholangiocarcinoma presented with acute respiratory distress due to massive pulmonary embolism.
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4/6. calciphylaxis associated with cholangiocarcinoma treated with low-molecular-weight heparin and vitamin K.

    calciphylaxis is a rare disorder of small-vessel calcification and cutaneous infarction associated with chronic renal failure. Rare cases of calciphylaxis not associated with chronic renal failure have been reported with breast cancer, hyperparathyroidism, and alcoholic cirrhosis. To our knowledge, we report the first case of calciphylaxis without chronic renal failure associated with cholangiocarcinoma and the first attempt to treat calciphylaxis with vitamin K. A 56-year-old woman presented with necrotic leg ulceration. She was treated initially with low-molecular-weight heparin, with no effect. A coagulation work-up showed vitamin k deficiency. During vitamin K therapy, the patient had fulminant progression of the calciphylaxis. She died, and an autopsy showed metastatic cholangiocarcinoma. thrombosis and protein c deficiency have been implicated in the pathophysiology of calciphylaxis. Functional protein c deficiency may be one of several factors contributing to the development of calciphylaxis. Vitamin K therapy was ineffective in our patient and may have been detrimental.
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5/6. pancreas-preserving biliary amputation with pancreatic diversion: a new surgical technique for complete resection of the intrapancreatic biliary system.

    Pancreatoduodenectomy is not optimal for organ preservation in patients with mucosal carcinoma of the choledochus. When the lesion spreads near the papilla of Vater, pancreas-preserving biliary amputation may be indicated to achieve complete resection of the biliary system. The first successful case is reported here with technical considerations. First, the pancreatic neck was divided and a tube was inserted into the main pancreatic duct beyond the papilla. The choledochus was dissected downward with division of the posterior pancreatoduodenal vessels. The main pancreatic duct was isolated with the aid of palpation of the tube, and was then ligated and divided. Subsequent dissection was performed to the level of the duodenal mucosa, which was incised circularly. The duodenal defect was then closed. The elevated jejunum was interposed between the pancreatic stumps and bilateral pancreaticojejunostomies were created. The procedure was successfully performed in a patient with superficially spreading cholangiocarcinoma. Postoperative bile leak and pancreatic fistula were controlled with medical management. The patient is currently well without tumor recurrence 19 months after surgery. Her glucose tolerance, which was moderately impaired preoperatively, has been maintained. pancreas-preserving biliary amputation has been developed as an organ-preserving procedure alternative to pancreatoduodenectomy. Indications, methods of pancreatic reconstruction, and long-term results require further study.
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6/6. Intrahepatic cholangiocarcinoma presenting as the budd-chiari syndrome: a case report and literature review.

    Intrahepatic cholangiocarcinoma, an increasingly recognized primary tumour of the liver, is associated with a very poor prognosis. A patient with this tumour who presented with budd-chiari syndrome (the first to the authors' knowledge in Western literature and only the third patient overall) secondary to extensive thrombosis in his inferior vena cava extending from the right atrium down to his iliac vessels is described. Neither curative nor palliative intervention was deemed to be an option in this patient, who deteriorated rapidly while on anti-coagulants. Postmortem examination confirmed the radiological findings, and histological analysis revealed characteristic appearances of this tumour within the biliary tree and invasion into the inferior vena cava. Furthermore, biliary dysplasia, which can be a precursor to this cancer, was also noted within some of the bile ducts.
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