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Current Status of Liver Transplantation for Cholangiocarcinoma

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Current Status of Liver Transplantation for Cholangiocarcinoma REVIEW ARTICLE GOLDARACENA, GORGEN, AND SAPISOCHIN Current Status of Liver Transplantation for Cholangiocarcinoma Nicolas Goldaracena, Andre Gorgen, and Gonzalo Sapisochin Multi-Organ Transplant, Division of General Surgery, Department of Surgery, University Health Network, University of Toronto, Toronto, Ontario, Canada Cholangiocarcinoma (CCA) is the second most common liver cancer, and it is associated with a poor prognosis. CCA can be divided into intrahepatic, hilar, and distal. Despite the subtype, the median survival is 12-24 months without treatment. Liver transplantation (LT) is recognized worldwide as a curative option for hepatocellular carcinoma. On the other hand, the initial results for LT for CCA were very poor mainly due to a lack of adequate patient selection. In the last 2 decades, improvements have been made in the management of unresectable hilar CCA, and the results of LT after neoadjuvant chemoradiation have been shown to be promising. This has prompted a consideration of hilar CCA as an indication for LT in some centers. Furthermore, some recent research has shown promising results after LT for patients with early stages of intrahepatic CCA. A better understanding of the best tools to prognosticate the outcomes of LT for CCA is still needed. Here, we aimed to review the role of LT for the treatment of patients with perihilar and intrahepatic CCA. Also, we will discuss the most recent advances in the field and the future direction of the management of this disease in an era of transplantation oncology. Liver Transplantation 24 294–303 2018 AASLD. Received July 18, 2017; accepted October 1, 2017. Since the establishment of liver transplantation (LT) stage liver disease, its indication to treat tumors con- as the best treatment option for patients with end- fined to the liver has grown substantially.(1,2) Hepatocellular carcinoma (HCC) is the most fre- quent liver cancer, and LT is the best treatment option in selected patients because it removes both the cancer Abbreviations: BILCAP, Adjuvant capecitabine for biliary tract can- cer; CA19-9, carbohydrate antigen 19-9; CCA, cholangiocarcinoma; (with the widest possible margins) and the underlying (3-5) ELITA, European Liver and Intestine Transplant Association; liver disease. However, as the understanding of HCC, hepatocellular carcinoma; hCCA, hilar cholangiocarcinoma; tumor biology and multidisciplinary oncological treat- HCC-CCA, mixed hepato-cholangiocarcinoma; HCV, hepatitis C virus; iCCA, intrahepatic cholangiocarcinoma; iCCA1HCC, concom- ments improve, the indication of LT to treat other itant intrahepatic cholangiocarcinoma and hepatocellular carcinoma in unresectable tumors confined to the liver (ie, cholangio- the explant; iCCA-HCC, mixed hepatocellular cholangiocarcinoma; carcinoma [CCA], metastatic neuroendocrine tumors) LT, liver transplantation; MELD, Model for End-Stage Liver Dis- (6-10) ease; OS, overall survival; PSC, primary sclerosing cholangitis; has expanded. LT has therefore gained momen- TRANSPHILL, Liver Resection Versus Radio-Chemotherapy- tum as a treatment for patients with cancer. Because of Transplantation for Hilar Cholangiocarcinoma; UCLA, University organ shortages, the main concern when offering a of California, Los Angeles; UNOS, United Network for Organ Shar- ing; VEGF, vascular endothelial growth factor. transplant to these patients is the impact on other patients on the waiting list.(11) With recent advances in Address reprint requests to Gonzalo Sapisochin, M.D., Multi- Organ Transplant, Division of General Surgery, Department of the treatment of hepatitis C virus (HCV), there seems Surgery, University Health Network, University of Toronto, 585 to be a decrease in the number of patients in need of a University Avenue, 11PMB184, Toronto, M5G 2N2, ON 1 1 LT because of HCV, resulting in a potential increase in Canada. Telephone: 1-416-340-5230; FAX: 1-416-340- (12) 5242; Email: [email protected] the organ pool. However, in some regions (such as North America), the increase in the incidence of Copyright VC 2017 by the American Association for the Study of Liver Diseases. patients with cirrhosis with nonalcoholic steatohepatitis may result in no change in the organ pool.(13) Further- View this article online at wileyonlinelibrary.com. more, living donor LT may be another excellent option DOI 10.1002/lt.24955 to provide access to LT to those patients with cancer. Potential conflict of interest: Nothing to report. In this regard, the use of LT for the treatment of CCA has become a focus of interest around the 294 | REVIEW ARTICLE LIVER TRANSPLANTATION, Vol. 24, No. 2, 2018 GOLDARACENA, GORGEN, AND SAPISOCHIN world.(14-16) This type of tumor arising from the bile duct epithelium is very aggressive, and its prognosis is mainly dependent on its location along the biliary tree and its chance of being completely resected with nega- tive margins.(17) The role of LT to treat patients with CCA has been explored in some centers for those patients presenting with an unresectable tumor confined to the hepatic hilum (ie, perihilar CCA/Klatskin tumor)(18-21) and more recently for patients with “very early” (single tumor 2 cm) intrahepatic cholangiocarcinomas (iCCAs).(8) So far, only these 2 locations of CCA are the ones where patients can potentially benefit from LT. Here we aimed to review the role of LT for the treat- ment of patients with perihilar CCA and iCCA. Also, FIG. 1. CCA locations. (A) Intrahepatic (iCCA). (B) Hilar we will discuss the most recent advances in the field and (hCCA). (C) Distal. the future direction of the management of this disease. CCA Classification approach was first attempted in the late 1990s and failed to show any promising results because the 5-year CCA is a malignant tumor of the biliary system that survival rate was reported to be approximately 18% stands as the second most common primary liver can- (Table 1).(33) However, this was a retrospective analysis (22) cer after HCC. More than 95% of the time, it con- of a small series where patients did not undergo any sists of an adenocarcinoma that depending on its type of protocolized neoadjuvant or adjuvant locations on the biliary tree, can be classified as iCCA treatment. or extrahepatic CCA.(23) In turn, the extrahepatic CCA can be subclassified into hilar (Klatskin tumor) or distal CCA (mid third and lower third bile duct THE “MAYO CLINIC” PROTOCOL (24) lesions; Fig. 1). In the year 2000, the Mayo Clinic group reported their preliminary experience with a protocol of neoadjuvant therapy followed by LT.(41) The neoadjuvant protocol Hilar CCA consisted of the combination of external beam and The incidence of hilar cholangiocarcinoma (hCCA; transcatheter radiation with intravenous 5-fluorouracil. Klatskin tumor) is approximately 7000 cases per year A total of 11 out of the initial 19 patients who were in North America, representing around two-thirds of found to be eligible for the study completed the proto- all cases of CCA.(25) Unfortunately, the overall survival col and underwent LT, showing encouraging (OS) for patients suffering from an hCCA is detri- results.(18) In 2004, the previous series was updated mental, ranging between 12 and 24 months.(26) and an 82% 5-year survival rate was reported for 24 It is well-known that the most important prognostic patients undergoing the “Mayo protocol.”(35,42) In factor is to achieve a complete resection with negative 2005, the same group published their experience of oncological margins, but unfortunately, this is only neoadjuvant chemoradiation followed by LT. In this achieved in 25%-40% of the patients.(27-29) The cur- study, they compared those patients undergoing a rent 5-year survival rate after surgery, even in select resection approach alone against those who underwent patients, rarely exceeds 40%.(30-32) From this low sur- neoadjuvant therapy in addition to LT.(10) The LT vival after resection derives the concept of treating this group with neoadjuvant chemoradiation (38 patients) patient population with a LT. This concept allows a achieved better OS at 1 year (92% versus 82%), 3 years complete resection with good negative margins in (82% versus 48%), and 5 years (82% versus 21%) when patients who have tumors that are locally unresectable compared with the resection group (26 patients). Also, due to the invasion of major vessels, bilobar tumor the LT group experienced lower posttransplant recur- involvement, or insufficient hepatic reserve. This rence (13% versus 27%).(10) Importantly, all the REVIEW ARTICLE | 295 GOLDARACENA, GORGEN, AND SAPISOCHIN LIVER TRANSPLANTATION, February 2018 TABLE 1. Patient Survival and Disease-Free Survival of Patients Transplanted With hCCA Neoadjuvant Number of Chemotherapy Total Patients 1- With or Number Who Year 5-Year Without of Underwent OS 3-Year OS Radiation References Center Study Type Patients LT (%) (%) OS (%) (%) Therapy Comments Iwatsuki et al.(34) University of Retrospective 72 38 (53) 60 32 25 61% 37 (51%) disease free (1998) Pittsburgh comparing of patients at 3 years resection to LT Sudan et al.(18) University of Retrospective 17 11 (65) — — — Yes Median survival (2002) Nebraska 11 months Rea et al.(10) Mayo Clinic Retrospective 71 38 (54) 79 61 58 Yes — (2005) comparing resection to LT Heimbach et al.(35) Mayo Clinic Retrospective 106 65 (61) 91 — 76 Yes Median time (2004) to recurrence 22 months Rosen et al.(36) Mayo Clinic Retrospective 148 90 (61) 82 63 55 Yes — (2008) Kaiser et al.(37) Germany Retrospective 47 47 (100) 61 31 22 No — (2008) Multicenter Darwish et al.(38) United States Retrospective 287 216 (75) — 68 (2 years) 53 Yes 65% 5-year (2012) Multicenter disease-free survival Welling et al.(20) University of Retrospective 12 6 (50) 83 — — Yes — (2014) Michigan Marchan et al.(39) Emory Retrospective 10 8 (80) 80 67% (2 years) — Yes — (2016) Mantel et al.(40) ELITA database Retrospective 173 28 (16)* — — 59* Yes This article compared (2016) Multicenter 77 (45)† 21† patient selection within “Mayo protocol” to beyond that protocol.
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