Cases reported "Cholangiocarcinoma"

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1/17. Combined hepatocellular-cholangiocarcinoma. Diagnostic challenge in hepatic fine needle aspiration biopsy.

    OBJECTIVE: To study the cytohistologic features of combined hepatocellular-cholangiocarcinoma (CHCC-CC) in fine needle aspiration biopsy (FNAB) material. STUDY DESIGN: Six hepatic FNAB cases with cell blocks (five) and hepatic resections (two) were analyzed cytohistologically and immunohistochemically. RESULTS: The six cases were diagnosed as CHCC-CC based on clinicopathologic correlation. Unequivocal hepatocellular carcinoma (HCC) cells corresponding to Edmondson and Steiner's grade 3 lesions were identified in the FNAB in three instances. adenocarcinoma, represented by cohesive columnar cells with ovoid, basal nuclei displaying nuclear palisading, acini and/or papillary structures with variable intracytoplasmic intraacinar or brush border mucin production, was identified in all cases. Intermediate cells with hybrid/polymorphic cytologic features straddling malignant hepatocytes and glandular cells were identified in five instances. Tissue alpha-fetoprotein was negative. There was brush border and/or diffuse cytoplasmic p-carcinoembryonic antigen immunoreactivity in the glandular elements. CONCLUSION: FNAB diagnosis of CHCC-CC is possible if the clinical, cytohistologic and immunohistochemical findings support the presence of HCC and adenocarcinoma. Intermediate cells pose a great challenge to recognize and define: they tend to lose the classic cytologic features of malignant hepatocytes and acquire glandular characteristics. At the very least, there should be a high index of suspicion. These cases underscore the necessity for clinicopathologic correlation in enhancing the precision of FNAB diagnoses.
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2/17. Pathologic fracture of the humerus due to metastatic cholangiocarcinoma.

    cholangiocarcinoma is a rare tumor of the bile duct system known to frequently metastasize to the axial skeleton, lungs, adrenal glands, brain, and lymphatic system. Spread to the long bones has not previously been reported in the literature partly because of the short life expectancies of patients with this disease. We discuss a case of a pathologic humerus fracture due to metastatic cholangiocarcinoma.
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3/17. Lymphoepithelioma-like cholangiocarcinoma: an Epstein-Barr virus-associated tumor.

    Epstein-Barr virus (EBV) has been linked to carcinomas of several body sites, especially of the nasopharynx, salivary gland, lung, and stomach. We present five cases of lymphoepithelioma-like cholangiocarcinoma, including one that had been previously reported. Two patients were men and three were women. Their ages ranged from 42 to 66 years. Histologically, all five tumors were composed of variable proportions of undifferentiated epithelial cells and glandular components in a lymphocyte-rich stroma. EBV was detected in all five tumors by in situ hybridization for EBER-1 in both lymphoepithelioma-like carcinoma (LELC) and glandular parts, but not in 36 cases of cholangiocarcinoma without the LELC component. Taken together, these observations indicate that lymphoepithelioma-like cholangiocarcinoma is strongly linked to EBV. The LELC type of cholangiocarcinoma, like LELC of other body sites, may be more common in areas with endemic EBV infection.
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4/17. Appraisal of intra-arterial infusion of prostaglandin E1 in patients undergoing major hepatic resection report of four cases.

    In order to reduce risk for postoperative acute liver failure, prostaglandin E1 (PGE1) was administered either from the hepatic artery (HA) or the superior mesenteric artery (SMA) in four high-risk cases undergoing major hepatic resection. Two cases were subjected to HA PGE1 infusion for 3 or 4 days after surgery at a rate of 0.01 microg/kg/min. Both patients had hepatocellular carcinoma (HCC) associated with chronic hepatitis, and ICG R15 was 17.6% and 14.5%, respectively. Right hemihepatectomy and extended right hemihepatectomy were performed. serum total bilirubin (T. Bil.) peak value was 2.2 mg/100 ml in Case 1 and 2.1 mg/100 ml in Case 2. In Case 1, decreased bile flow was observed immediately after cessation of PGE1. The other two cases were subjected to SMA PGE1 infusion for 5 or 6 days after surgery at the same rate. In Case 3, right hemihepatectomy was performed for HCC on a cirrhotic liver four weeks after right portal vein embolization, in which preoperative ICG R15 was 19.0%. Peak T. Bil level was 3.7 mg/100 ml with uneventful postoperative course. In Case 4 with a huge cholangioma, right trisegmentectomy was performed. Peak serum T. Bil level was 1.7 mg/100 ml in this uneventful postoperative course. In Case 3 and Case 4, portal blood flow, measured by Doppler ultrasonography, was markedly increased by PGE1 infusion. From these results, intra-arterial PGE1 infusion might be useful in prevention of postoperative liver failure after major hepatic resection.
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5/17. Intestinal type cholangiocarcinoma of intrahepatic large bile duct associated with hepatolithiasis--a new histologic subtype for further investigation.

    Intestinal metaplasia is regarded as a possible predisposing factor of cancer, particularly of the intestinal type adenocarcinoma. The clinicopathologic features of intestinal type adenocarcinoma have been well documented in the stomach, and intestinal metaplasia and intestinal type adenocarcinoma has also been reported in the gallbladder. However, regarding the intrahepatic bile ducts, the clinicopathologic features are not yet clear and there have been no reports in English literature on intestinal type intrahepatic cholangiocarcinoma. We report a case of intestinal type cholangiocarcinoma associated with hepatolithiasis in the large intrahepatic bile duct. The tumor showed mainly intraductal papillary growth primarily composed of absorptive columnar cells. Particularly, Paneth cell metaplasia of carcinoma cells was widespread, and goblet cells and neuroendocrine cells were also observed in the carcinoma tissue, to a varied degree. It showed an intraluminal spread along the dilated intrahepatic ducts with minimal ductal stromal invasion. In the vicinity of the tumor, intestinal metaplasia was also identified in the adjacent hyperplastic and dysplastic bile duct epithelium. Some bile ducts contained stones and the mural glands of the bile ducts showed hyperplastic change secondary to stones. This case is considered to provide the evidence supporting the concept of the metaplasia-dysplasia-carcinoma sequence via intestinal metaplasia in the stone-containing intrahepatic bile ducts.
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6/17. Primary liver cancer with dual expression of hepatocyte and bile duct epithelial markers.

    A 65-year-old Japanese man with chronic hepatitis c was found to have a hepatic tumor by ultrasonography. Both dynamic computed tomography and hepatic angiography showed a hypervascular tumor with a central defect. Since a diagnosis of hepatocellular carcinoma was made, transcatheter arterial embolization was performed. However, the tumor metastasized to systemic lymph nodes and the patient died 3 months after treatment. An autopsy was performed. Histologic examination of the hepatic tumor revealed that the peripheral part was completely necrotic and the central area was composed of strands of pleomorphic cells with focal gland formation surrounded by fibrosis. No production of mucin or bile was evident. The microscopic findings of metastatic lymph nodes were similar to those of the central portion of the hepatic tumor. Immunohistochemical strains of the hepatic tumor and lymph node metastases showed diffuse positivity for cytokeratins 7 and 19, while hepatocyte paraffin 1 was focally reactive. These findings suggest that the hepatic tumor was a combined hepatocellular carcinoma and cholangiocarcinoma. Since the tumor expressed dual phenotypic markers of both hepatocytes and bile duct cells, the tumor might have an intermediate phenotype between hepatocytes and bile duct cells.
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7/17. Combined hepatocellular and cholangiocarcinoma with marked squamous cell carcinoma components arising in non-cirrhotic liver.

    We report a surgical case of liver tumor, 40 x 35 mm in size, with squamous cell carcinoma (SCC) and hepatocellular carcinoma (HCC) components in a 60-year-old Japanese man with steatohepatitis. Most of the SCC component showed typical intercellular bridge and keratinization, while most of the HCC components showed a thick trabecular pattern with mild to moderate nuclear atypia. Both components transit each other without undifferentiated foci; however, a small foci showing glandular structure was intermediated. No cyst formation was found in the liver. The primary site of the squamous cell carcinoma was not detected in general clinical and radiological examination. Immunohistochemical analysis revealed that part of the HCC components neighboring the SCC showed patchy and weak expression of cytokeratin 7. There are several possibilities for the origin of squamous cell carcinoma in this case: marked squamous metaplastic change of cholangiocarcinoma and/or HCC, and carcinoma originating from pleuripotential stem cells. Irregular fatty changes, scattered giant mitochondria and acellular fibrosis with bridging were seen in the liver; however, this patient had no episode of hepatitis-associated viral infection. This is an interesting case of combined hepatocellular and cholangiocarcinoma with marked SCC components arising in a non-cirrhotic fibrotic liver.
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8/17. Bile duct cancer developing 21 years after choledochoduodenostomy.

    BACKGROUND: cholangiocarcinoma in patients with choledochoenterostomy has been a rare condition with few cases reported in the literature written in English, and there have been few pathological descriptions of this disease. However, it has recently been documented as a late complication of choledochoenteric anastomosis, and has become a serious issue today since choledochoenterostomy and sphincteroplasty for benign biliary lesions were frequently resorted to in the 1970s and 1980s. The authors here present a case of a 67-year-old woman who developed a cholangiocarcinoma 21 years after choledochoduodenostomy. methods: The patient underwent a curative resection of the tumor with a hepatopancreatoduodenectomy after a detailed preoperative assessment, including cholangiography and cholangioscopy. RESULTS: Although this tumor has been reported to have a poor prognosis, a precise preoperative evaluation and aggressive surgery may contribute to better survival. Pathologically, the tumor originated in the bile duct mucosa of the choledochoduodenostomy, and was accompanied by intestinal and pyloric gland-like metaplasia. CONCLUSION: These epithelial changes presumably induced by chronic inflammation due to the reflux of intestinal contents and bile stasis may be responsible for the carcinogenesis.
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9/17. Rhabdoid cholangiocarcinoma: a variant of cholangiocarcinoma with aggressive behavior.

    A rhabdoid cholangiocarcinoma is a very rare variant of sarcomatous cholangiocarcinomas. Here, we report a vimentin positive cholangiocarcinoma showing rhabdoid features in the entire tumor, with a very aggressive behavior. A 41-year-old woman was admitted to our hospital due to a huge hepatic mass. The resected liver revealed a 17 x 15 cm sized solid mass with extensive necrosis and an infiltrative border. On microscopic examination, the entire tumor was composed of loosely cohesive round to polygonal cells, with rhabdoid features having abundant eosinophilic glassy cytoplasm and eccentrically located vesicular nuclei. Some tumor cells contained intracytoplasmic mucin vacuoles, but definite areas of glandular differentiation or spindle cell were not found. Immunohistochemical staining showed a diffuse strong positive reaction to pan-cytokeratin and vimentin, and focal positivity for the carcinoembryonic antigen. Other immunohistochemical stainings for cytokeratin 7, cytokeratin 20, S-100 protein, HMB-45, desmin, alpha-smooth muscle actin, c-kit, CD34, alpha-fetoprotein, anti-hepatocyte antigen, chromogranin and synaptophysin were all negative. After two months, the patient developed a local recurrence along the resection margin, and multiple hematogenous metastases to the lung and liver were seen on the follow-up CT scan.
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10/17. Intrahepatic cholangiocarcinoma with lymphoepithelioma-like carcinoma component.

    We report an unusual case of intrahepatic cholangiocarcinoma (ICC) with lymphoepithelioma-like carcinoma (LELC) component in a 60-yr-old woman who was found incidentally to have an abdominal mass. Histologically, the tumor showed 2 distinct patterns with dense lymphoplasma cell infiltration. The first pattern, comprising approximately 20% of total tumor volume, showed the features of lymphoepithelioma-like carcinoma, as commonly found in nasopharyngeal carcinoma (NPC). The second pattern was a moderately differentiated cholangiocarcinoma. In situ hybridization for Epstein-Barr virus (EBV)-encoded rna (EBER) showed positive nuclear labeling of tumor cells in both patterns, but not in surrounding inflammatory cells. By the polymerase chain reaction, the latent membrane protein gene (LMP-1) in this case was shown to have a 30 bp deletion in the C-terminus, a unique feature in high prevalence areas of undifferentiated nasopharyngeal carcinoma, such as in taiwan. Presence of the EBV genomes and their expression in the cholangiocarcinoma cells suggested that EBV may play an important role in the pathogenesis of ICC with LELC. In this case, it is unclear why only 20% of the glands were transformed into LELC. The mechanism whereby EBV transforms the malignant glands into the distinct morphology resembling NPC warrants further investigation.
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