Cases reported "Biliary Tract Neoplasms"

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1/6. Hepatobiliary cystadenoma with mesenchymal stroma in a patient with chronic hepatitis c.

    Hepatobiliary cystadenoma was suggested to be uncommon and it is often difficult to make a differential diagnosis. We report a case of a 65-year-old woman who presented with changes in the structure of a cyst that had been observed for the previous 10 years. diagnostic imaging revealed a 7-cm-diameter cystic lesion with internal septations and papillary projections in her liver. All laboratory test results were normal; however, cystic fluid carcinoembryonic antigen (CEA) and carbohydrate antigen (CA) 19-9 were raised, at 160 ng/ml andover 200,000 U/ml, respectively. Owing to changes in the structure of the cyst and the difficulty of differential diagnosis from cystadenocarcinoma, a complete surgical excision was performed. The histological findings indicated that the tumor consisted of a multilocular cyst lined by glandular cells (with cuboidal or tall columnar cystoplasm), which were immunohistochemically positive for cytokeratin, CEA, epithelial membrane antigen, and CA 19-9. The underlying stroma was composed of proliferating primitive spindle cells which were immunoreactive for vimentin, alpha-smooth muscle actin, muscle-specific actin, and desmin, and resembled ovarian stroma. From these findings, this tumor was diagnosed as hepatobiliary cystadenoma with mesenchymal stroma. Even though the tumor was previously diagnosed as a simple liver cyst, it was necessary to pay special attention to the changes in the structure of the cyst, using ultra sonography and/or computed tomography, bearing in mind hepatobiliary cystadenoma with mesenchymal stroma. The malignant potential of this tumor is stressed, and complete surgical resection is the recommended therapy.
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2/6. Oncocytic biliary cystadenocarcinoma: a case report and review of the literature.

    We report an unusual case of biliary cystadenocarcinoma with oncocytic differentiation. The patient was a 43-year-old woman who presented with right upper quadrant pain. Imaging revealed a 16 x 10 x 10-cm, heterogenous, right hepatic mass with extension into the right atrium. Surgical resection revealed a papillary neoplasm of malignant cells with atypical hyperchromatic nuclei and prominent nucleoli lining fibrovascular cores. Mesenchymal stroma was not present. The majority of the epithelial cells had abundant eosinophilic granular cytoplasm, consistent with oncocytic differentiation. There was extensive stromal and hepatic parenchymal invasion. Immunohistochemical staining revealed a "biliary pattern" of cytokeratin subset immunoreactivity, with positivity for cytokeratin 7 and an absence of staining with cytokeratin 20. The tumor was negative for mucin, carcinoembryonic antigen, alpha-fetoprotein, calretinin, CD31, and chromogranin. There was granular cytoplasmic staining with phosphotungstic acid hematoxylin, consistent with the presence of abundant mitochondria. Electron microscopy revealed abundant mitochondria within the neoplastic cells. This case is quite unusual because female patients only rarely lack the characteristic ovarian-like mesenchymal stroma of biliary cystadenomas/cystadenocarcinomas. Furthermore, to our knowledge, oncocytic differentiation in this neoplasm has been reported previously on only 2 occasions. The biologic behavior and prognostic significance, if any, of the lack of mesenchymal stroma in female patients or the presence of oncocytic differentiation remains to be further elucidated as more of these cases are described.
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3/6. Hepatobiliary cystadenoma with mesenchymal stroma: expression of oestrogen receptors in formalin-fixed tissue.

    We report three cases of hepatobiliary cystadenoma with mesenchymal stroma in which oestrogen receptor immunostaining was carried out, after using a microwave method of antigen retrieval. In one of the tumours immunoreactivity for oestrogen receptors was demonstrated within the mesenchymal stromal cells. The presence of oestrogen receptors supports the theory that oestrogens act as tumour promoters and may explain the female predilection.
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4/6. Second cancer during long-term survival after resection of biliary tract carcinoma.

    Curative surgery for biliary tract malignancy has improved the prognosis of patients; however, during long-term follow up after extensive surgery, four of our patients (two with gallbladder carcinoma and two with bile duct carcinoma) developed a second primary cancer (one each in the duodenum, skin, descending colon, and lung). Regular examination of the upper gastrointestinal tract, colorectum, and lungs, and testing for tumor markers (carcinoembryonic antigen and CA19-9) were performed as follow-up studies. As a result, the second cancers were all found at a relatively early stage and all four patients are still alive 6-14 years after the initial operation. A review of the annual autopsy reports over the last 5 years in japan showed that the incidence of second cancer was 14.8% in patients with gallbladder carcinoma and 13.5% in those with bile duct carcinoma. Furthermore, the rate of second gastric and colorectal carcinoma was significantly higher in patients with primary bile duct cancer than in those with primary gallbladder cancer. In conclusion, the incidence of a second cancer after resection of biliary tract malignancy is more than 10%, but this second cancer can be detected relatively easily and treated at an early stage during the course of regular long-term follow up for the first cancer.
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5/6. Mucinous biliary cystadenoma with mesenchymal stroma: expressions of CA 19-9 and carcinoembryonic antigen in serum and cystic fluid.

    A case of mucinous biliary cystadenoma with mesenchymal stroma (CMS tumor) in a 64-year-old woman is reported. The patient presented with acute abdominal pain and a palpable mass in the upper abdomen. Computed tomography and abdominal sonography showed characteristic multilocular cysts in the left lobe of the liver. serum CA 19-9 was elevated to 108 U/ml with normal carcinoembryonic antigen (CEA) and alpha-fetoprotein (AFP) levels. The levels of CA 19-9 and CEA in the cystic fluid were high at 7430 U/ml and 576 ng/ml, respectively. The serum CA 19-9 returned to 35 U/ml 4 weeks after tumor resection. These corresponding findings of both tumor markers in the serum and cystic fluid imply that (1) CA 19-9 and CEA both exist in the epithelial component of CMS tumors as evidenced by immunohistochemical stain, (2) serum CA 19-9 is a valuable marker in the diagnosis and monitoring of CMS, and (3) in cystic fluid, there are more significantly high levels of CA 19-9 in CMS compared with levels in simple cyst and polycystic liver disease. Therefore, measurement of CA 19-9 in cystic fluid and serum may be helpful in the differential diagnosis of hepatic cystic lesions.
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6/6. An unusual etiology of biliary hilar obstruction and the potential role of acidic fibroblast growth factor in the development of a biliary neuroma.

    neuroma of the biliary tract is a rare condition thought to be caused by trauma secondary to cholecystectomy. More rare is the occurrence that causes symptomatic biliary obstruction. A 65-year-old woman was hospitalized because of abdominal pain, nausea, vomiting, and general malaise of 1 to 2 months duration. cholecystectomy had been performed 40 years before. Ultrasound revealed hepatomegaly and dilated intrahepatic ducts. CT showed intra- and extrahepatic ductal dilatation with questionable intraductal mass. Endoscopic retrograde cholangiopancreatography and percutaneous transhepatic cholangiography demonstrated stricture of the hepatic duct bifurcation. The biliary bifurcation was resected, and hepaticojejunostomy was performed. The patient's postoperative course was unremarkable. Histological examination of the surgical specimen revealed positive staining for the S-100 antigen of the obstructing luminal stricture (without evidence of cholangiocarcinoma), which was consistent with a biliary neuroma. Positive staining was also found for acidic (and not basic) fibroblast growth factor (FGF) and two of its high affinity receptors (FGFR-1 and FGFR-4). This study supports the apparent association between biliary neuromas and cholecystectomy as well as the potential role of an established angiogenic and neurogenic growth factor in the formation of this tumor. Finally, this case is also unique in that it represents the longest interval between cholecystectomy and presentation of a biliary neuroma, 40 years after surgery.
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