The rate of cholangiocarcinoma in Caroli Disease - A German multicenter study,HPB

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The rate of cholangiocarcinoma in Caroli Disease - A German multicenter study
HPB ( IF 2.7 ) Pub Date : 2021-07-07 , DOI: 10.1016/j.hpb.2021.06.423
Mohammad H Fard-Aghaie ₁ , Georgios Makridis ₁ , Tim Reese ₁ , Bernd Feyerabend ₂ , Kim C Wagner ₁ , Andreas Schnitzbauer ₃ , Wolf O Bechstein ₃ , Felix Oldhafer ₄ , Moritz Kleine ₄ , Jürgen Klempnauer ₄ , Jens Rolinger ₅ , Silvio Nadalin ₅ , Alfred Königsrainer ₅ , Nikolaos Vassos ₆ , Robert Grützmann ₆ , Tamas Benkö ₇ , Andreas Paul ₇ , Jun Li ₈ , Lutz Fischer ₈ , Katharina Beaumont ₉ , Natascha Nüssler ₉ , René Fahrner ₁₀ , Utz Settmacher ₁₀ , Stefan Fichtner-Feigl ₁₁ , Wenzel Schöning ₁₂ , Johann Pratschke ₁₂ , Jennifer A Eckhoff ₁₃ , Roger Wahba ₁₃ , Christiane Bruns ₁₃ , Alexander Bernsmeier ₁₄ , Felix Braun ₁₄ , Thomas Becker ₁₄ , Georg Lurje ₁₅ , Ulf P Neumann ₁₆ , Jonas Dohmen ₁₇ , Steffen Manekeller ₁₇ , Jörg C Kalff ₁₇ , Arianeb Mehrabi ₁₈ , Markus W Büchler ₁₈ , Sven A Lang ₁₉ , Hans J Schlitt ₂₀ , Verena Tripke ₂₁ , Hauke Lang ₂₁ , Karl J Oldhafer ₁

Affiliation

Department of Surgery, Division of Hepatobiliary and Pancreatic Surgery, Asklepios Hospital Barmbek, Hamburg, Germany; Semmelweis University of Medicine, Medical Faculty, Asklepios Campus Hamburg, Hamburg, Germany.
MVZ Hanse Histologikum, Hamburg, Germany.
Department of General and Visceral Surgery, Frankfurt University Hospital, Goethe-University Frankfurt/Main, Frankfurt/Main, Germany.
Department of General, Abdominal and Transplant Surgery, Hannover Medical School, Hannover, Germany.
Department of General, Visceral and Transplant Surgery, University Hospital Tübingen, Tübingen, Germany.
Department of Surgery, University Hospital of Erlangen, Erlangen, Germany.
Department of General, Visceral and Transplantation Surgery, University Hospital Essen, Essen, Germany.
Department of Visceral Transplant Surgery, University Medical Center Hamburg Eppendorf, Hamburg, Germany.
Department of General, Visceral and Endocrine Surgery, München Klinik Neuperlach, Munich, Germany.
Department of General, Visceral and Vascular Surgery, University Hospital Jena, Jena, Germany.
Department of General and Visceral Surgery, Medical Centre and Faculty of Medicine University of Freiburg, Freiburg, Germany.
Department of Surgery, Charité - Universitätsmedizin Berlin, Campus Charité Mitte, Campus Virchow Klinikum, Berlin, Germany.
Department of General, Visceral, Cancer and Transplantation Surgery, University Hospital of Cologne, Cologne, Germany.
Department of General, Visceral-, Thoracic-, Transplantation- and Pediatric Surgery, University Medical Center Schleswig-Holstein (UKSH), Campus Kiel, and Christian-Albrecht University (CAU), Kiel, Germany.
Department of Surgery, Charité - Universitätsmedizin Berlin, Campus Charité Mitte, Campus Virchow Klinikum, Berlin, Germany; Department of Surgery and Transplantation, University Hospital RWTH Aachen, Aachen, Germany.
Department of Surgery and Transplantation, University Hospital RWTH Aachen, Aachen, Germany.
Department of Surgery, University of Bonn, Bonn, Germany.
Department of Surgery, University of Heidelberg, Heidelberg, Germany.
Department of Surgery and Transplantation, University Hospital RWTH Aachen, Aachen, Germany; Department of Surgery, University Hospital Regensburg, Regensburg, Germany.
Department of Surgery, University Hospital Regensburg, Regensburg, Germany.
Department of General, Visceral and Transplant Surgery, Universitätsmedizin Mainz, Mainz, Germany.

Background

Caroli Disease (CD) and Caroli Syndrome (CS) are rare disorders presenting with dilation of the intrahepatic bile ducts. CD/CS are associated with cholangiocarcinoma (CCA). However, the true incidence of CCA is still unclear, although it may serve as an indication for surgery. In this paper, we analyzed (I) the incidence of CCA in German centers, (II) reviewed our single center population together with its clinical presentation and (III) performed a thorough literature review.

Methods

17 large HPB-centers across Germany were contacted and their patients after surgical treatment due to CD/CS with histopathology were included. Medline search for all studies published in English or German literature was performed. Patients who underwent surgery at our department between 2012 and 2020 due to CD or CS were analyzed.

Results

In the multicenter study, 79 patients suffered from CD and 119 patients from CS, with a total number of 198 patients. In 14 patients, CCA was found (Overall: 7,1%; CD: 6,3%, CS 7,6%). Between 2012 and 2020, 1661 liver resections were performed at our department. 14 patients underwent surgery due to CD or CS. Histological examination showed synchronous cholangiocarcinoma in one patient.

The literature review revealed a CCA-rate of 7,3% in large series, whereas in case reports a rate of 6,8% was found.

Discussion

There is risk of malignant transformation and patients with CD might also benefit from resection due to improvement of symptoms. Therefore, resection is strongly advised.

As certain patients with CS require transplantation, treatment should not be guided by the relatively low rate of CCA but by the concomitant diseases that come along with hepatic failure.

中文翻译：

Caroli 病中胆管癌的发病率——一项德国多中心研究

背景

Caroli 病 (CD) 和 Caroli 综合征 (CS) 是罕见的疾病，表现为肝内胆管扩张。CD/CS 与胆管癌 (CCA) 相关。然而，CCA 的真实发病率仍不清楚，尽管它可以作为手术的指征。在本文中，我们分析了 (I) 德国中心的 CCA 发病率，(II) 回顾了我们的单中心人群及其临床表现，以及 (III) 进行了彻底的文献回顾。

方法

联系了德国的 17 个大型 HPB 中心，并纳入了因 CD/CS 和组织病理学而接受手术治疗的患者。对发表在英语或德语文献中的所有研究进行 Medline 搜索。分析了2012年至2020年间因CD或CS在我科接受手术的患者。

结果

在多中心研究中，79 例 CD 患者和 119 例 CS 患者，总计 198 例患者。在 14 名患者中发现了 CCA（总体：7.1%；CD：6.3%，CS 7.6%）。2012 年至 2020 年间，我科共进行了 1661 例肝切除术。14 名患者因 CD 或 CS 接受了手术。组织学检查显示一名患者同时患有胆管癌。

文献回顾显示，大型系列的 CCA 率为 7.3%，而在病例报告中，发现率为 6.8%。