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| 1 | Risk of malignancy in Caroli disease and syndrome: A systematic review显示文摘BACKGROUND Congenital intrahepatic bile duct dilatation without fibrosis is called Caroli disease(CD),and is called Caroli syndrome(CS)when it has fibrotic and cirrhotic liver morphology.The development of intrahepatic carcinoma is described in both conditions,but the reported incidence varies extensively.Potential risk factors for the malignant transformation were not described.Furthermore,conservative or surgical treatment is performed depending on the extent of cystic malformation,hepatic dysfunction and structural hepatic changes,but little is known about which treatment should be offered to patients with CD or CS and cancer.AIM To further investigate the malignant transformation in these conditions.METHODS A systematic review of the current literature until January 2019 was performed according to the Preferred Reporting Items for Systematic Reviews and Meta-Analyses statement.A search using Medline(PubMed)was performed using a combination of Medical Subject Headings terms“caroli disease”,“caroli syndrome”,“tumor”,“malignant”,and“cholangiocarcinoma”.Only human studies published in English were used for this systematic review.The following parameters were extracted from each article:year of publication,type of study,number of patients,incidence of malignant tumor,duration of symptoms,age,sex,diagnostics,identification of tumor,surgical therapy,survival and tumor recurrence.RESULTS Twelve retrospective studies reporting the courses of 561 patients(53%females)were included in this systematic review.With a mean age of 41.6 years old(range 23 to 56 years old),patients were younger than other populations undergoing liver surgery.Depending on the size of the study population the incidence of cholangiocarcinoma varied from 2.7%to 37.5%with an overall incidence of 6.6%.There were only few detailed reports about preoperative diagnostic work-up,but a multimodal work-up including ultrasound of the liver,computed tomography,magnetic resonance imaging and endoscopic retrograde cholangiopancreatography was used in most studies.Disease duration was variable with up to several years.Most patients had episodes of cholangitis,sepsis,fever or abdominal pain.Tumor detection was an incidental finding of the surgical specimen in most cases because it is currently often impossible to detect tumor manifestation during preoperative diagnostics.Liver resection or liver transplantation was performed depending on the extent of the biliary pathology and additional alterations of the liver structure or function.No postoperative adjuvant chemotherapy was reported,but chemotherapy was administered in selected cases of tumor recurrence.Overall survival rates after one year were low at 36%and a high recurrence rate of up to 75%during the observation period.CONCLUSION Only few retrospective studies reported a low tumor incidence.Despite the high rate of mortality and tumor recurrence,definite surgical treatment should be offered as soon as possible. | Rene Fahrner Sandra GC Dennler Daniel Inderbitzin | 2020 | World Journal of Gastroenterology2020，26，31： | 2 |
| 2 | 查看详情显示文摘 | Lungenschmieda Christoph Dennler Gilles Neugebauer Helmut | | 0，，： | 1 |
| 3 | Transforming growth factor beta signal transduction显示文摘 | Dennler S Goumans MJ Peter TD | 2002 | J Leukoc Biol2002，71，5： | 1 |
| 4 | Direct binding of Smad3 and Smad4 to critical TGF-B inducible elements in the promoter of human plasaninogen activator inhibitor-type I gene显示文摘 | Dennler S Itoh S Vivien D | 1998 | EMBO J1998，17，11： | 1 |
| 5 | A short aminoacid sequence in MH 1 domain is responsible for functional differences between Smad2 and Smad3显示文摘 | Dennler S Huet S Gauthier JM | 1999 | Oncogene1999，18，8： | 1 |
| 6 | c-Jun inhibits transforming growth factor beta-mediated transcription by repressing Smad 3 transcriptional activity显示文摘 | Dennler S Prunier C Ferrand N | | 0，，： | 1 |
| 7 | TGF-beta/SMAD/GLI2 signaling axis in cancer progression and metastasis显示文摘 | Javelaud D Alexaki VI Dennler S | 2011 | Cancer Res2011，71，17： | 1 |
| 8 | Flexi- ble, long-lived, large-area, organic solar cells 显示文摘 | Lungensehmied C Dennler G Neugebauer H | 2007 | Sol En- ergy Mater Sol Ceils2007，91，： | 1 |
| 9 | Transforming growth factor beta signal transduction 显示文摘 | Dennler S Goumans M J Peter TD | 2002 | J Leukoc Biol2002，71，5： | 1 |
| 10 | Polymer Fullerene Bulk-Heterojunction Solar Cells 显示文摘 | DENNLER GILLES SCHARBER MARKUS C BRABEC CHRISTOPH | 2009 | J AdvMater2009，21，： | 1 |
| 11 | Transforming growth fac tot beta signal transduction显示文摘 | Dennler S Goumans MJ Peter TD | 2002 | J Leukocyte Biol2002，71，5： | 1 |
| 12 | Design Rules for Donors in Bulk-Heterojunction Tandem Solar Cells- Towards 15 % Energy-Conversion Efficiency 显示文摘 | Gilles Dennler Markus C Scharber Tayebeh Ameri | 2008 | Advanced Materials2008，20，3： | 1 |
| 13 | Flexible coniugated polymer- based plastic solar cells: From basics to applications 显示文摘 | Dennler G Sariciftch N S | 2005 | IEEE2005，93，： | 1 |
| 14 | TGF-β/SMAD/GLI2signaling axis in cancer progression and metastasis显示文摘 | Javelaud D Alexaki VI Dennler S | | 0，，17： | 1 |
| 15 | Polymer-fullerene bulk-heterojunction solar ceils 显示文摘 | DENNLER G SCHARBER M C BRABEC C J | 2009 | Adv Mater2009，21，： | 1 |
| 16 | Transforming growth factor beta signal transduction显示文摘 | Dennler S Goumans MJ Peter TD | 2002 | Leukoc Biol2002，71，5： | 1 |
| 17 | Direct binding of Smad3 and Smad4 to critical TGF beta-inducible elements in the promoter of human plasminogen activator inhibitor-type 1 gene显示文摘 | Dennler S Itoh S Vivien D | 1998 | EMBO J1998，17，11： | 1 |
| 18 | Performance improvement of organic solar cells with moth eye anti-reflection coating显示文摘 | Karen Forberich Gilles Dennler | 2008 | Thin Solid Films2008，516，： | 1 |
| 19 | Induction of Sonic Hedgehog mediators by transforming growth factor-beta: Smad 3-dependent activation of Gli 2 and Gli 1 expression in vitro and in vivo 显示文摘 | Dennler S Andre J Alexaki I | 2007 | Cancer Res2007，67，： | 1 |
| 20 | Flexible conjugated polymerbasedplastic solar cells : from basics to applications显示文摘 | Dennler G Sariciftci N S | 2005 | Proc IEEE2005，93，8： | 1 |
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