Cases reported "Adenoma, Bile Duct"

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1/11. Atypical bile duct adenoma, clear cell type: a previously undescribed tumor of the liver.

    A variable proportion of bile duct adenomas of the liver are still confused with metastatic well-differentiated adenocarcinoma by surgeons and pathologists. We present here three examples of previously undescribed primary hepatic bile duct tumors that were composed almost entirely of clear cells that closely mimicked metastatic renal cell carcinoma. They were interpreted as atypical bile duct adenomas and occurred in two males and one female whose ages ranged from 25 to 64 years. All three tumors were incidental findings and measured from 0.8 to 1.1 cm. The clear neoplastic cells showed mild nuclear atypia and no mitotic activity. They were arranged in tubules and nests that focally infiltrated the hepatic parenchyma. For comparison, a case of clear cell cholangiocarcinoma and 13 conventional bile duct adenomas were examined. The clear cell cholangiocarcinoma was larger (6.0 cm) and had the tubular pattern of conventional cholangiocarcinoma and an abundant desmoplastic stroma. The clear cells of this tumor exhibited greater nuclear atypia and increased mitotic activity. All three atypical bile duct adenomas expressed cytokeratin (CK) 7, p53 protein, epithelial membrane antigen (EMA), and carcinoembryonic antigen (CEA); they were negative for CK20, vimentin, Hep Par 1, chromogranin, and prostatic specific antigen (PSA) and exhibited less than 10% of Ki-67-positive nuclei. One atypical bile duct adenoma displayed luminal immunoreactivity for villin. With the exception of Ki-67 reactivity, the 13 conventional bile duct adenomas and the clear cell cholangiocarcinoma had essentially a similar immunohistochemical profile as that of the atypical clear cell bile duct adenomas. The absence of an extrahepatic primary tumor, the histologic features, the immunohistochemical profile, and the fact that all patients are symptom-free 2 months to 18 years after wedge liver biopsy support the interpretation of atypical clear cell bile duct adenoma. The differential diagnosis with clear cell hepatocellular carcinoma and metastatic clear cell carcinomas is discussed.
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2/11. Extensive portal tumor thrombi with portal hypertension in an autopsy case of intrahepatic cholangiocarcinoma.

    Vascular invasion is not a prominent feature of cholangiocarcinoma (CCC), in contrast to hepatocellular carcinoma (HCC), which frequently shows extensive vascular tumor thrombi. We report an autopsy case of CCC with extensive portal tumor thrombi and portal hypertension. A 57-yr-old man presented with abdominal pain. liver imaging revealed no tumors, but showed intrahepatic portal venous obstruction. HCC with portal tumor thrombi was suspected clinically. His clinical course was rapid; he died of hepatic failure 50 days after admission. At autopsy, the liver (2,700 g) was studded with diffuse whitish yellow granular areas with flecks of coalescent granules. Intrahepatic portal veins were diffusely occluded by tumor thrombi. Microscopically, the tumor was poorly differentiated adenocarcinoma with mucin; tumor cells were immunohistochemically positive for carcinoembryonic antigen, CA 19-9, DU-PAN-2, and biliary type cytokeratins, but negative for alpha-fetoprotein. Tumor cells were diffuse in the liver, and there were numerous tumor thrombi in the small portal veins. hepatic veins and small arteries were occasionally occluded by tumor thrombi. There was ascites, splenomegaly and tumor thrombi in the gastric and esophageal veins, suggesting that portal hypertension had been present. This tumor seemed to have marked affinity to invade portal veins. It must be stressed that there are CCCs with extensive portal tumor thrombi and resultant portal hypertension.
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3/11. Intrahepatic cholangiocarcinoma with sarcomatous transformation: an autopsy case.

    We report an adult case of intrahepatic cholangiocarcinoma with remarkable sarcomatous changes. At autopsy a yellowish-white tumor (8 x 6 cm) was found in the left hepatic lobe, and there were several daughter nodules in both hepatic lobes. Histologically, most of the main tumor, and all of the daughter nodules examined, showed sarcomatous changes (spindle- or fusiform-shaped and pleomorphic cells). Histologic examination of a whole slice of the main tumor disclosed a focus of adenosquamous carcinoma (cholangiocarcinoma) within the tumor. Frequent transitions between adenosquamous carcinoma areas and sarcomatous areas suggested that sarcomatous transformation occurred in cholangiocarcinoma and then grew to spread rapidly. Immunohistochemically, squamous carcinoma and, to a lesser degree, adenocarcinoma elements were strongly positive for keratin and epithelial membrane antigen, both being also weakly positive in sarcomatous cells, supporting that possibility. vimentin was positive only in sarcomatous elements. cholangiocarcinoma should be included in the list of hepatic tumors showing sarcomatous change.
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4/11. A case of diffuse type of combined hepatocellular and cholangiocellular carcinoma initially presented as a localized small nodule.

    A combined hepatocellular and cholangiocellular carcinoma of diffuse type in a Japanese man is described. A small localized solitary tumor apparently grew rapidly into a diffuse-type carcinoma, and the liver weight increased about 4-fold during the last two months. The clinical course of this case was as expected for a diffuse type of hepatocellular carcinoma except that unusually high levels of serum carcinoembryonic antigen were found. The patient died of hepatic failure with systemic bleeding five months later. At autopsy, multiple small nodules were suspected to be intrahepatic metastatic foci because portal tumor thrombus was observed in the right antero-superior segment where the initial tumor was localized. Histologically, the tumor had components of both hepatocellular and mucin-producing cholangiocellular carcinoma. This is believed to be the first report on a diffuse type of combined hepatocellular and cholangiocellular carcinoma initially presented as a localized small nodule.
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5/11. Combined hepatocellular-cholangiocarcinoma associated with dermatomyositis.

    A 56 year old female developed combined hepatocellular cholangiocarcinoma associated with dermatomyositis. serum tumour markers except for carbohydrate antigen (CA 19-9; 6400 ng/ml) were within normal range. Despite extensive chemotherapy, no clinical response was obtained and the patient's course deteriorated after 4 months. Macroscopically, the liver was mainly occupied by hepatocellular carcinoma but cholangiocarcinoma was found in the hilum. This is the first case of a rare association of combined hepatocellular-cholangiocarcinoma and dermatomyositis.
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6/11. Combined hepatocellular and cholangiocarcinoma arising in a cirrhotic liver. Report of an autopsy case.

    A rare case of combined hepatocellular and cholangiocarcinoma arising in a 56-year-old female is reported. The autopsy disclosed the presence of two different kinds of tumors in the right lobe of the liver, which showed advanced cirrhosis; a massive rubbery, ill-defined and whitish-yellow cholangiocarcinoma and a nodular soft, encapsulated and dark green hepatocellular carcinoma. They were adjacent to each other, but showed no intermingling. Only the massive cholangiocarcinoma had invaded the portal vein and showed several intrahepatic metastatic foci and hepatic, pancreaticoduodenal and perigastric lymph node metastases. Immunohistochemically, carbohydrate antigen 19-9 was strongly positive only for the cholangiocarcinoma component, explaining the high titer of this antigen in the serum on admission. On the basis of these findings, the possible morphogenesis of the tumor observed in the cirrhotic liver is discussed.
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7/11. Clinical aspects of intrahepatic bile duct carcinoma including hilar carcinoma: a study of 57 autopsy-proven cases.

    The clinical features of 57 autopsied cases of intrahepatic bile duct carcinoma including 28 cases of the peripheral type (cholangiocarcinoma in the narrow sense) and 29 cases of the hilar type are described in comparison with those of hepatocellular carcinoma, with a review of the literature on the clinicopathological aspects of intrahepatic bile duct carcinoma. As compared with hepatocellular carcinoma, the average age of the patients was older; the male predominance was not obvious, chronic parenchymal liver disease was infrequent in the past history, association of primary cirrhosis was seldom, cholestatic features were frequently the early signs and more pronounced during the course, the liver was enlarged to a lesser extent, ascites was less common, signs of portal hypertension were absent or minimal, and extrahepatic metastases were less frequent. In many respects, the hilar type resembled extrahepatic bile duct carcinoma, and the peripheral type was somewhat between it and hepatocellular carcinoma. Although the overall survival was not much different from that for hepatocellular carcinoma, early diagnosis is emphasized; this would make surgical management possible. Differential diagnosis from hepatocellular carcinoma may be possible in the majority with direct cholangiography, liver scan, celiac angiography, determination of alpha-fetoprotein and hepatitis b antigen, and blood chemistry such as SGOT, SLDH, serum bilirubin and alkaline phosphatase. Illustrative cases are given including one patient with a hilar carcinoma who survived for more than 2 years after transhepatic biliary drainage.
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8/11. Hepatobiliary cystadenoma.

    Hepatobiliary cystadenomas are rare, truly benign cystic epithelial tumors with the potential to become cystadenocarcinoma. The initial symptom is vague, nonspecific abdominal pain, frequently in middle-aged women, but patients may be asymptomatic. Abdominal ultrasonography may be diagnostic. Intracystic fluid, obtained by sonographically guided fine-needle aspiration, may contain elevated levels of carcinoembryonic antigen. Complete resection or enucleation is the treatment of choice. We present the cases of two patients with hepatobiliary cystadenoma.
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9/11. Biliary cystadenoma of the liver with elevated CA 19-9.

    A case of intrahepatic biliary cystadenoma with mesenchymal stroma is reported. The tumor was associated with strikingly elevated serum and intracystic levels of the tumor-associated antigen CA19-9. Two months after surgical enucleation, serum CA 19-9 levels returned to normal.
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10/11. Rapidly growing mucinous cholangiocarcinoma.

    A 65-year-old man was hospitalized due to an abdominal tumor. Several imaging studies showed multilocular tumors in the right hepatic lobe and in the pancreatic head, 4 and 7 cm in size, respectively. The hepatic tumor rapidly grew to 9 cm in 4 weeks, associated with a rapid increase in the serum carcinoembryonic antigen level from 125 ng/ml to 1,000 ng/ml. The pathologic diagnosis of the resected liver tumor was mucinous cholangiocarcinoma. This hepatic tumor produced a large amount of mucin, but did not secrete mucin into the bile ducts. Therefore, there was no obstructive jaundice. The incidentally accompanied pancreatic tumor was a typical serous cystadenoma. The present case suggested that mucinous cholangiocarcinoma could present rapid growth of the tumor. The clinicopathological features of mucinous cholangiocarcinoma are demonstrated for the first time, and the differences between mucinous cholangiocarcinoma and mucin-producing papillary adenocarcinoma of duct-spreading type are discussed.
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