Cases reported "Cholangiocarcinoma"

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1/4. 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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2/4. Intrahepatic cholangiocarcinoma with lymphoepithelioma-like component.

    We present two cases of intrahepatic cholangiocarcinoma with lymphoepithelioma-like component. The patients included one woman and one man, aged 67 and 41 years, respectively. They presented with right upper quadrant pain and epigastralgia. Histologically, both tumors showed two distinct histological patterns with dense lymphoplasma cell infiltration. The first pattern was a well to moderately differentiated adenocarcinoma; the second component showed a feature similar to lymphoepithelioma-like carcinoma. Granulomatous reaction was noted in one case. Immunohistochemical study revealed that both tumors were immunoreactive with AE1/AE3, cytokeratin 7, and cytokeratin 19 but negative for carcinoembryonic antigen and cytokeratin 20. The stromal lymphocytes were composed of predominantly CD3( ) T cells. in situ hybridization for Epstein-Barr virus (EBV)-encoded rna (EBER) showed positive nuclear signal in tumor cells but not in inflammatory cells in one case. The presence or absence of EBV genome was confirmed by polymerase chain reaction of LMP-1 gene in both cases. The LMP-1 gene also had a 30-bp deletion in Exon 3 as compared with the products from B95-8 cells. We further sequenced the PCR product and confirmed a 30-bp deletion between Nucleotide (nt) 168,282 and nt 168,253 corresponding to the B95-8 sequence. The clinical significance of 30-bp deletion in Exon 3 of the LMP-1 gene in lymphoepithelioma-like carcinoma of the liver warrants further investigation.
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3/4. 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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4/4. A combined hepatocellular/cholangiocellular carcinoma with sarcomatoid features.

    A case of primary liver cancer showing combined hepatocellular and cholangiocellular features and an associated pseudosarcomatous (schwannomatous) component is presented. Histologically, compact microtrabecular and glandular patterns in a dense fibrous stroma were recognized. The latter showed transition towards spindle cell sarcomatous growth in several areas of both the primary and the lymph-node metastasis. Glandular areas expressed acidic mucins and AE1-reactive keratins; albumin mRNA was detected by in situ hybridization in both trabecular and glandular areas. vimentin and S-100 protein were mostly expressed in the pseudosarcomatous areas. Both the morphological patterns and the phenotypic features indicate that divergent differentiation along both epithelial and mesenchymal lineages took place in this rare primary liver tumor.
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