Gene/Protein Disease Symptom Drug Enzyme Compound
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Query: UMLS:C0023890 (cirrhosis)
 42,195 document(s) hit in 31,850,051 MEDLINE articles (0.00 seconds)

Von-Meyenburg complexes (VMC) are seen frequently in the liver and are largely considered to be innocuous, with only 11 cases reported in the literature of neoplastic transformation of VMCs. The authors report three cases of cholangiocarcinoma, each occurring in a background of fibrosis and nodularity that was reported initially as micronodular cirrhosis. Although the livers showed cirrhosis, the central veins were often preserved, and regenerative activity was patchy and focal. Histologic examination revealed many VMCs, and a gradual transition from VMCs to hyperplastic or adenomatous lesions and cholangiocarcinoma. The adenomatous lesions consisted of extensive replacement of the parenchyma by tumor-like nodules of ductular proliferations without obvious features of malignancy. All three patients were older than 60 years of age and had portal hypertension. Computed tomographic scans showed multiple, small renal cysts in one patient. Immunohistochemical staining showed positivity for epithelial membrane antigen, carcinoembryonic antigen, and keratins (AE1/AE3 and CAM5.2) in tumor cells, consistent with cholangiocarcinoma. The pattern of fibrosis and nodularity in these cases is not typical of either congenital hepatic fibrosis or usual cirrhosis. The authors propose that these patients represent another aspect in the spectrum of ductal plate malformations that may be modified by other factors such as alcohol, drugs, or infection. To their knowledge, neoplastic transformation of VMCs in the background of such changes has never been reported before.
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PMID:Evidence for the neoplastic transformation of Von-Meyenburg complexes. 1093 54

This is a report of a post-mortem histological, histochemical, and immunohistochemical examination of a rare case of sclerosing encapsulating peritonitis (SEP) and non-occlusive mesenteric infarction (NOMI), two serious complications of continuous ambulatory peritoneal dialysis (CAPD), with which a man suffering hepatitis C virus (HCV)-induced liver cirrhosis for 7 years and trauma-induced paraplegia for 50 years had been treated for 1 year. The direct cause of death was encephalopathy caused by extreme hyperammonemia (11 250 microg/dL in serum). The autopsy revealed that the SEP had drastically reduced the length of the small intestine to 210 cm, 180 cm of which presented acute ischemic enteritis with Gram-negative bacterial infection. Histological examination of the SEP revealed that the exterior was composed of normal serosal elastic lamina, but with a cocoon-like appearance remarkably thickened by fibrosis to 3-8 times that of the normal subserosal layer and consisting of spindle cells and blood vessels, with some infiltration of mast cells and lymphocytes. The immunohistochemical examination of the spindle cells revealed few AE1/AE3(+) cells, HHF35(+) cells, and CD34(+) cells, many CD117(+) cells with slight proliferative activity based on MIB-1 positivity (proliferation index <1%), but no CD44(+) cells. It was concluded that either the few CD34(+) and/or the many CD117(+) cells were mesenteric stem cells that had originated from the serosa, proliferated, then differentiated into myofibroblasts or fibroblasts, producing collagen and hyaluronic acid in the matrix, leading to the gradual formation of the SEP, which was induced by the continual irritation of CAPD.
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PMID:Sclerosing encapsulating peritonitis and non-occlusive mesenteric infarction found at autopsy in a man who had undergone continuous ambulatory peritoneal dialysis: a histochemical and immunohistochemical study. 1097 66

A 65-year-old man was referred to our hospital for treatment of a liver tumor. Abdominal ultrasonography (US) demonstrated a low echoic mass in the S2-S4 region of the liver, which was confirmed by abdominal computed tomography (CT). In the delayed phase of angio-CT, the inside of the mass was not enhanced. Abdominal angiography showed a hypovascular area in the liver. An extended left lobectomy was performed. Macroscopically, the tumor was 9.5 x 9.5 cm in size, and on cross section, it was white and clearly demarcated from the surrounding tissue. Microscopic observation of hematoxylin-eosin-stained specimens did not show any glandular or trabecular formation. Histologically, there was diffuse proliferation of atypical spindle cells that had hyperchromatic, short, spindle-shaped nuclei, and pale cytoplasm with poor intercellular adhesion. The nontumorous tissue was almost normal with no sign of cirrhosis. Immunohistochemical examination showed that the spindle cells were positive for vimentin and cytokeratins (AE1/AE3, CAM 5.2), but negative for all other markers. The final diagnosis was a sarcomatoid carcinoma, the origin of which was not able to be confirmed immunohistochemically. This case of a primary hepatic tumor composed of malignant cells with sarcomatous features is described, and the immunohistochemical findings are discussed.
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PMID:Malignant sarcomatoid tumor of the liver: report of a case. 1129 15

Lymphoepithelioma-like carcinomas (LELC) of the liver are rare. Only nine cases have been reported. All of them were considered to be cholangiocarcinoma and the majority were positive for Epstein-Barr virus (EBV) on EBER in situ hybridization. Here we report a case of hepatocellular carcinoma (HCC) mainly composed of LELC. The patient was a 56-year-old man with chronic hepatitis C virus (HCV) infection and cirrhosis. A right-side hepatectomy was performed to remove a 3-cm diameter tumor. Microscopically, the tumor was mainly composed of undifferentiated carcinoma with heavy lymphocytic infiltration, consistent with LELC. The tumor cells of the LELC component were focally positive for HePar 1, CK19 and CK7 and more diffusely positive (50% of tumor cells) for AE1/AE3 on immuno-histochemical study. EBER in situ hybridization was negative. This is the first confirmed case of HCC with an LELC component. In the available literature, all three cases of LELC of the liver that were negative for EBV were associated with chronic viral hepatitis and cirrhosis, suggesting a different carcinogenesis of EBV-positive LELC of the liver.
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PMID:Lymphoepithelioma-like hepatocellular carcinoma. 1759 7

Reported herein is an unusual case of intrahepatic cholangiocarcinoma with lymphoepithelioma-like appearance in a 64-year-old man who was found to have an intrahepatic mass without cirrhosis. The tumor had two distinct histological patterns with dense lymphoplasmacytic infiltrate. The first was similar to nasopharyngeal undifferentiated carcinoma; the second pattern was a well-differentiated adenocarcinoma. Transition between the two components was observed in the same duct. Immunohistochemistry indicated that the tumor was immunoreactive with AE1/AE3 and cytokeratin (CK) 7, but negative for CEA and CK20. Stromal inflammatory infiltrate primarily consisted of plasma cells and lymphocytes. Immunohistochemical examination and in situ hybridization for EBV showed no integration of the virus in the tumor cells. Intrahepatic lymphoepithelioma-like carcinoma is rare, and most are associated with EBV. Only three cases were not associated with EBV. The authors would like to add one more example of the tumors not associated with EBV.
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PMID:Lymphoepithelioma-like cholangiocarcinoma not associated with EBV. 1806 45

Lymphoepithelioma-like cholangiocarcinomas are rare tumors and most of them are related with Epstein-Barr virus (EBV) infection. Here, a case of a patient with lymphoepithelioma-like cholangiocarcinoma not associated with EBV infection is presented. In a 79-year-old man with hepatitis B virus-associated cirrhosis, a liver mass was detected on abdominal CT. Macroscopically, the resected tumor was pale gray, rubbery and well defined. Histologically, the tumor was composed of two components: an adenocarcinoma that formed irregular small glands and a lymphoepithelioma-like carcinoma that exhibited sheets of undifferentiated epithelial cells with lymphoid stroma. Lymphoplasmacytic infiltrates were more predominant in the lymphoepithelioma-like carcinoma than in the adenocarcinoma. Both components were roughly divided, but they gradually merged. Immunohistochemically, the adenocarcinoma component was diffusely positive for AE1/AE3, cytokeratin 7, cytokeratin 19 and epithelial membrane antigen, while the lymphoepithelioma-like carcinoma component was focally positive for them. However, both components were diffusely positive for p53 protein, and in situ hybridization using EBV-encoded RNA 1 was negative in both components as well. Examination of a resected para-aortic lymph node revealed metastasis exclusively of the lymphoepithelioma-like carcinoma component.
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PMID:Intrahepatic lymphoepithelioma-like cholangiocarcinoma not associated with epstein-barr virus: a case report. 2147 93