Cholangiocarcinoma:A 7-year experience at a single center in Greece

AIM: To evaluate survival rate and clinical outcome of cholangiocarcinoma.METHODS: The medical records of 34 patients with cholangiocarcinoma,seen at a single hospital between the years 1999-2006,were retrospectively reviewed.RESULTS: Thirty-four patients with a median age of 75 years were included.Seventeen (50%) had painless jaundice at presentation.Sixteen (47.1%) were perihilar,15 (44.1%) extrahepatic and three (8.8%) intrahepatic.Endoscopic retrograde cholangiography (ERCP) and/or magnetic resonance cholangiography (MRCP) were used for the diagnosis.Pathologic confirmation was obtained in seven and positive cytological examination in three.Thirteen patients had co-morbidities (38.2%).Four cases were managed with complete surgical resection.All the rest of the cases (30) were characterized as non-resectable due to advanced stage of the disease.Palliative biliary drainage was performed in 26/30 (86.6%).The mean follow-up was 32 mo (95% CI,20-43 mo).Overall median survival was 8.7 mo (95% CI,2-16 mo).The probability of 1-year,2-year and 3-year survival was 46%,20% and 7%,respectively.The survival was slightly longer in patients who underwent resection compared to those who did not,but this difference failed to reach statistical significance.Patients who underwent biliary drainage had an advantage in survival compared to those who did not (probability of survival 53% vs 0% at 1 year,respectively,P = 0.038).CONCLUSION: Patients with cholangiocarcinoma were usually elderly with co-morbidities and/or advanced disease at presentation.Even though a slight amelioration in survival with palliative biliary drainage was observed,patients had dismal outcome without resection of the tumor.

Modern diagnostic approaches to cholangiocarcinoma

BACKGROUND:Cholangiocarcinoma is a very aggressive tumor with poor survival.Therefore,early diagnosis and surgical resection are of paramount importance.Its diagnosis is difficult because access to the tumor is not easy.Biopsy is possible only for intrahepatic cholangiocarcinoma,which accounts for 10% of cases.Routine brush cytology from endoscopic retrograde cholangiopancreatography(ERCP) has a high specificity of 100% but unfortunately a low sensitivity of 30%.In this review we briefly describe new diagnostic techniques applicable to ERCP brush cytology specimens and targeting the genetic background of the disease,in particular fluorescence in situ hybridization(FISH) and digital image analysis(DIA).DATE SOURCES:The PubMed database up to 2011 was used for the retrieval of relevant articles.The search terms FISH,fluorescence in situ hybridization,DIA,digital image analysis and cholangiocarcinoma were used.Both original and review articles were used.RESULTS:FISH identifies cells with chromosomal abnormalities,mainly numerical aberrations,using a mixture of fluorescencelabeled probes.FISH offers a higher sensitivity than routine cytology,retaining a high level of specificity.The DIA criterion for malignancy is demonstration of aneuploidy.This technique increases the sensitivity to 40%,but the specificity remains low.Preliminary data from application to other tumors suggest that combination of FISH and DIA may be of further benefit.CONCLUSIONS:The new techniques offer a significantly enhanced diagnostic efficacy in the evaluation of ERCP brush specimens.Apart from contributing to a more timely diagnosis,their wider application to cholangiocarcinoma may also facilitate the genetic study of the disease and add to our understanding of oncogenesis at the molecular level,with the prospect of identifying targets for novel therapeutic interventions.

Successful treatment of noncirrhotic portal hypertension with eculizumab in paroxysmal nocturnal hemoglobinuria: A case report

BACKGROUND Idiopathic non-cirrhotic portal hypertension(INCPH)is mainly associated with thrombophilia in Western countries.Paroxysmal nocturnal hemoglobinuria(PNH)is a rare hematologic disease that manifests with hemolytic anemia,thrombosis,and peripheral blood cytopenias.Portal and hepatic venous thrombosis were reported in PNH.A rare case of INCPH complicating PNH is described.CASE SUMMARY A 63-year old woman with a 2-year past medical history of PNH without treatment was admitted because of jaundice and refractory ascites requiring large volume paracentesis.Liver histology revealed portal venopathy with portal fibrosis and sclerosis,nodular regenerative hyperplasia,parenchymal ischemic changes,and focal sinusoidal and perivenular fibrosis without bridging fibrosis or cirrhosis,all indicative of INCPH.The flow cytometry confirmed PNH diagnosis and eculizumab treatment was initiated.Her condition was improved gradually,bilirubin was normalized 6 months following initiation of eculizumab,and 1 year later diuretics were stopped.CONCLUSION Eculizumab improved intravascular hemolysis and reversed clinical manifestations of INCPH in a patient with paroxysmal nocturnal hemoglobinuria.

Translation and validation of the Greek chronic liver disease questionnaire

AIM:To translate into Greek and validate the chronic liver disease questionnaire (CLDQ).METHODS:Two hundred and six consecutive adult patients with the diagnosis of a chronic liver disease from 2 general hospitals in Athens were enrolled in the study from May to September 2008.In order to assess their quality of life (QOL) the CLDQ was applied.The instrument was translated from English,back translated and reviewed in focus groups within the framework of a large multicenter study.The measurements that were performed included:2 independent sample t tests,oneway analysis of variance,reliability coefficients,explanatory factor analysis using a varimax rotation and the principal components method.RESULTS:One hundred and twenty five (61%) patients were men,half were aged 40-59 years and > 33% were > 60 years old.Among the patients,48 (23%) were hospitalized and 97 (47%) were cirrhotic according to the Child-Pugh score.The internal consistency of the Greek CLDQ version using Cronbach's alpha coefficient was found to be 0.93.Exploratory factor analysis identified 7 domains accounting for 65% of the variance of CLDQ items and only partially overlapping with those found in the original version.The area under the receiver operating characteristics curve was calculated at 0.813 and the logistic estimate for the threshold score of 167.50 provided a sensitivity of 74.3% and a specificity of 71.6% for the model.CONCLUSION:Our data confirmed the validity of the Greek version of the CLDQ in identifying the QOL among patients with chronic liver disease.