Z Gastroenterol 2024; 62(01): 50-55
DOI: 10.1055/a-2221-6126
Kasuistik

Laparoscopic Living donor liver transplantation in irresectable intrahepatic cholangiocarcinoma in primary sclerosing cholangitis associated liver cirrhosis

Leberlebendspende beim technisch irresektablen intrahapatischen Cholangiokarzinom in Leberzirrhose bei primär sklerosierender Cholangitis
Jan Bednarsch
1   Department of Surgery and Transplantation, University Hospital RWTH Aachen, Aachen, Germany (Ringgold ID: RIN39058)
2   Department of Surgery and Transplantation, University Hospital Essen, Essen, Germany (Ringgold ID: RIN39058)
,
Sven A. Lang
1   Department of Surgery and Transplantation, University Hospital RWTH Aachen, Aachen, Germany (Ringgold ID: RIN39058)
2   Department of Surgery and Transplantation, University Hospital Essen, Essen, Germany (Ringgold ID: RIN39058)
,
Daniel Heise
1   Department of Surgery and Transplantation, University Hospital RWTH Aachen, Aachen, Germany (Ringgold ID: RIN39058)
2   Department of Surgery and Transplantation, University Hospital Essen, Essen, Germany (Ringgold ID: RIN39058)
,
Pavel Strnad
3   Department of Internal Medicine III, University Hospital RWTH Aachen, Aachen, Germany
,
Ulf P. Neumann
1   Department of Surgery and Transplantation, University Hospital RWTH Aachen, Aachen, Germany (Ringgold ID: RIN39058)
2   Department of Surgery and Transplantation, University Hospital Essen, Essen, Germany (Ringgold ID: RIN39058)
,
Tom F. Ulmer
1   Department of Surgery and Transplantation, University Hospital RWTH Aachen, Aachen, Germany (Ringgold ID: RIN39058)
2   Department of Surgery and Transplantation, University Hospital Essen, Essen, Germany (Ringgold ID: RIN39058)
› Author Affiliations

Abstract

Intrahepatic cholangiocarcinoma (iCCA) is the second most common primary liver tumor and usually associated with a poor oncological prognosis. The current gold standard is the surgical resection of the tumor with subsequent adjuvant therapy. However, in case of irresectability e.g. in case of liver cirrhosis, a palliative treatment regime is conducted.

This report demonstrates the case of an irresectable iCCA in liver cirrhosis due to primary sclerosing cholangitis (PSC) treated by living-donor liver transplantation (LDLT) facilitated by minimal invasive donor hepatectomy. No postoperative complications were observed in the donor and the donor was released on the 6th postoperative day. Further, after a follow-up of 1.5 years, no disease recurrence was detected in the recipient.

According to the recent international literature, liver transplantation can be evaluated in case of small solitary iCCA (< 3 cm) in cirrhosis. Less evidence is provided for transplantation in advanced tumors which are surgically not resectable due to advanced liver disease or infiltration of major vessels, however some reports display adequate long-term survival after strict patient selection. The selection criteria comprise the absence of distant metastases and locoregional lymph node metastases as well as partial remission or stable disease after neoadjuvant chemotherapy. Due to no established graft allocation for iCCA in Germany, LDLT is currently the best option to realize transplantation in these patients. Developments in the last decade indicate that LDLT should preferentially be performed in minimal invasive manner (laparoscopic or robotic) as this approach is associated with less overall complications and a shorter hospitalization.

The presented case illustrates the possibilities of modern surgery and the introduction of transplant oncology in the modern therapy of patients combining systemic therapy, surgical resection and transplantation to achieve optimal long-term results in patients which were initially indicated for palliative treatment.

Zusammenfassung

Das intrahepatische Cholangiokarzinom (iCCA) ist der zweithäufigste primäre Lebertumor und ist mit einer schlechten onkologischen Prognose vergesellschaftet. Die chirurgische Resektion mit anschließender adjuvanter Chemotherapie stellt die Therapie der Wahl dar. Bei Irresektabilität u.a. aufgrund des Vorliegens einer Leberzirrhose wird üblicherweise eine palliative Therapie eingeleitet.

Diese Kasuistik berichtet über einen Patienten mit einem irresektablen iCCA bei Zirrhose im Rahmen einer primär sklerosierenden Cholangitis (PSC), der nach neoadjuvanter Chemotherapie einer Lebertransplantation mittels minimalinvasiver Lebendspende zugeführt wurde. Beim Spender zeigten sich keine Komplikationen und eine kurze Hospitalisierungszeit. Der Empfänger zeigt im mittelfristigen Langzeitverlauf nach 1,5 Jahren kein Hinweis auf Rezidiv.

Nach aktueller, internationaler Literaturlage kann die Transplantation von kleinen, solitären iCCA (< 3 cm) in Zirrhose erwogen werden. Weniger Evidenz liegt für den Fall der technischen Irresektabilität bei fortgeschritteneren Befunden vor. Nichtsdestotrotz gibt es Hinweise auf gute Langzeitergebnisse nach strikter Selektion dieser Patienten. Dies umfasst den Ausschluss von Fernmetastasierung und loko-regionärer Lymphknotenmetastasen sowie zumindest Befundstabilität unter vorangehender Systemtherapie. Aufgrund aktuell fehlender Allokationsmöglichkeiten bei iCCA im Standardverfahren stellt die Lebendspende die beste Möglichkeit zur Realisierung einer Transplantation dar. Die Ergebnisse der letzten Dekade zeigen, dass diese präferenziell minimalinvasiv (laparoskopisch oder robotisch) aufgrund einer geringeren Komplikationsrate und kürzerer Hospitalisierung durchgeführt werden sollte.

Der vorgestellte Fall zeigt die Möglichkeiten der modernen Chirurgie und die Entwicklung der Transplantationsonkologie, in der (onkologischen) Therapie, chirurgische Resektionen und Transplantation inklusive Lebendspende eingesetzt werden, um optimale Langzeitergebnisse bei vormals palliativ eingestuften Patienten zu erzielen.



Publication History

Received: 03 September 2023

Accepted after revision: 03 December 2023

Article published online:
09 January 2024

© 2024. Thieme. All rights reserved.

Georg Thieme Verlag KG
Rüdigerstraße 14, 70469 Stuttgart, Germany

 
  • Reference

  • 1 Banales JM, Marin JJG, Lamarca A. et al. Cholangiocarcinoma 2020: the next horizon in mechanisms and management. Nature reviews Gastroenterology & hepatology 2020; 17 (09) 557-588 DOI: 10.1038/s41575-020-0310-z. (PMID: 32606456)
  • 2 Mavros MN, Economopoulos KP, Alexiou VG. et al. Treatment and Prognosis for Patients With Intrahepatic Cholangiocarcinoma: Systematic Review and Meta-analysis. JAMA surgery 2014; 149 (06) 565-574
  • 3 Primrose JN, Fox RP, Palmer DH. et al. Capecitabine compared with observation in resected biliary tract cancer (BILCAP): a randomised, controlled, multicentre, phase 3 study. The lancet oncology 2019; 20 (05) 663-673
  • 4 Bednarsch J, Czigany Z, Heij LR. et al. Compelling Long-Term Results for Liver Resection in Early Cholangiocarcinoma. J Clin Med 2021; 10 (13) 2959 DOI: 10.3390/jcm10132959. (PMID: 34209368)
  • 5 Vithayathil M, Khan SA. Current epidemiology of cholangiocarcinoma in Western countries. Journal of hepatology 2022; 77 (06) 1690-1698 DOI: 10.1016/j.jhep.2022.07.022. (PMID: 35977611)
  • 6 Bednarsch J, Czigany Z, Lurje I. et al. The role of ALPPS in intrahepatic cholangiocarcinoma. Langenbeckʼs archives of surgery/Deutsche Gesellschaft fur Chirurgie 2019. DOI: 10.1007/s00423-019-01838-2
  • 7 Mizuno T, Ebata T, Yokoyama Y. et al. Combined Vascular Resection for Locally Advanced Perihilar Cholangiocarcinoma. Annals of surgery 2022; 275 (02) 382-390 DOI: 10.1097/SLA.0000000000004322. (PMID: 32976284)
  • 8 Heimbach JK, Gores GJ, Haddock MG. et al. Liver transplantation for unresectable perihilar cholangiocarcinoma. Seminars in liver disease 2004; 24 (02) 201-207 DOI: 10.1055/s-2004-828896. (PMID: 15192792)
  • 9 Darwish Murad S, Kim WR, Harnois DM. et al. Efficacy of neoadjuvant chemoradiation, followed by liver transplantation, for perihilar cholangiocarcinoma at 12 US centers. Gastroenterology 2012; 143 (01) 88-98
  • 10 Goldstein RM, Stone M, Tillery GW. et al. Is liver transplantation indicated for cholangiocarcinoma?. American journal of surgery 1993; 166 (06) 768-771 DOI: 10.1016/s0002-9610(05)80696-8. (PMID: 8273866)
  • 11 Sapisochin G, Facciuto M, Rubbia-Brandt L. et al. Liver transplantation for “very early” intrahepatic cholangiocarcinoma: International retrospective study supporting a prospective assessment. Hepatology 2016; 64 (04) 1178-1188
  • 12 European Association for the Study of the Liver. Electronic address eee, European Association for the Study of the L: EASL-ILCA Clinical Practice Guidelines on the management of intrahepatic cholangiocarcinoma. Journal of hepatology 2023; 79 (01) 181-208
  • 13 Bowlus CL, Arrive L, Bergquist A. et al. AASLD practice guidance on primary sclerosing cholangitis and cholangiocarcinoma. Hepatology 2023; 77 (02) 659-702 DOI: 10.1002/hep.32771. (PMID: 36083140)
  • 14 Gross S, Bitzer M, Blodt S. et al. S3 Guideline: Diagnostic and therapy of hepatocellular carcinoma and biliary tract cancer. Zeitschrift fur Gastroenterologie 2023; 61 (04) e157-e171
  • 15 Diaz-Gonzalez A, Vilana R, Bianchi L. et al. Thermal Ablation for Intrahepatic Cholangiocarcinoma in Cirrhosis: Safety and Efficacy in Non-Surgical Patients. Journal of vascular and interventional radiology : JVIR 2020; 31 (05) 710-719 DOI: 10.1016/j.jvir.2019.06.014. (PMID: 31685360)
  • 16 Lunsford KE, Javle M, Heyne K. et al. Liver transplantation for locally advanced intrahepatic cholangiocarcinoma treated with neoadjuvant therapy: a prospective case-series. Lancet Gastroenterol Hepatol 2018; 3 (05) 337-348
  • 17 McMillan RR, Javle M, Kodali S. et al. Survival following liver transplantation for locally advanced, unresectable intrahepatic cholangiocarcinoma. American journal of transplantation : official journal of the American Society of Transplantation and the American Society of Transplant Surgeons 2022; 22 (03) 823-832 DOI: 10.1111/ajt.16906. (PMID: 34856069)
  • 18 Oh DY, He AR, Qin S. et al. Durvalumab plus Gemcitabine and Cisplatin in Advanced Biliary Tract Cancer. NEJM Evidence 2022; 1 (08) EVIDoa2200015 DOI: 10.1016/S2468-1253(22)00043-7. (PMID: 35278356)
  • 19 Wassmer CH, El Hajji S, Papazarkadas X. et al. Immunotherapy and Liver Transplantation: A Narrative Review of Basic and Clinical Data. Cancers (Basel) 2023; 15 (18) 4574 DOI: 10.3390/cancers15184574. (PMID: 37760542)
  • 20 Woo SM, Kimchy AV, Sequeira LM. et al. Immunotherapy Use Prior to Liver Transplant in Patients with Hepatocellular Carcinoma. Current oncology 2022; 29 (12) 9813-9825
  • 21 Lurje G, Bednarsch J, Czigany Z. et al. The prognostic role of lymphovascular invasion and lymph node metastasis in perihilar and intrahepatic cholangiocarcinoma. European journal of surgical oncology : the journal of the European Society of Surgical Oncology and the British Association of Surgical Oncology 2019; 45 (08) 1468-1478 DOI: 10.1016/j.ejso.2019.04.019. (PMID: 31053477)
  • 22 Rauchfuss F, Ali-Deeb A, Rohland O. et al. Living Donor Liver Transplantation for Intrahepatic Cholangiocarcinoma. Current oncology 2022; 29 (03) 1932-1938 DOI: 10.3390/curroncol29030157. (PMID: 35323357)
  • 23 Cherqui D, Soubrane O, Husson E. et al. Laparoscopic living donor hepatectomy for liver transplantation in children. Lancet 2002; 359 (9304) 392-396 DOI: 10.1016/S0140-6736(02)07598-0. (PMID: 11844509)
  • 24 Soubrane O, Perdigao Cotta F, Scatton O. Pure laparoscopic right hepatectomy in a living donor. American journal of transplantation : official journal of the American Society of Transplantation and the American Society of Transplant Surgeons 2013; 13 (09) 2467-2471
  • 25 Soubrane O, Eguchi S, Uemoto S. et al. Minimally Invasive Donor Hepatectomy for Adult Living Donor Liver Transplantation: An International, Multi-institutional Evaluation of Safety, Efficacy and Early Outcomes. Annals of surgery 2022; 275 (01) 166-174 DOI: 10.1097/SLA.0000000000003852. (PMID: 32224747)
  • 26 Zhao X, Lei Z, Gao F. et al. Minimally invasive versus open living donors right hepatectomy: A systematic review and meta-analysis. International journal of surgery 2021; 95: 106152 DOI: 10.1016/j.ijsu.2021.106152. (PMID: 34688930)
  • 27 Mu C, Chen C, Wan J. et al. Minimally Invasive Donors Right Hepatectomy versus Open Donors Right Hepatectomy: A Meta-Analysis. J Clin Med 2023; 12 (08) 2904 DOI: 10.3390/jcm12082904. (PMID: 37109241)
  • 28 Yeow M, Soh S, Starkey G. et al. A systematic review and network meta-analysis of outcomes after open, mini-laparotomy, hybrid, totally laparoscopic, and robotic living donor right hepatectomy. Surgery 2022; 172 (02) 741-750 DOI: 10.1016/j.surg.2022.03.042. (PMID: 35644687)
  • 29 Taketomi A, Kayashima H, Soejima Y. et al. Donor risk in adult-to-adult living donor liver transplantation: impact of left lobe graft. Transplantation 2009; 87 (03) 445-450 DOI: 10.1097/TP.0b013e3181943d46. (PMID: 19202452)
  • 30 Iida T, Ogura Y, Oike F. et al. Surgery-related morbidity in living donors for liver transplantation. Transplantation 2010; 89 (10) 1276-1282 DOI: 10.1097/TP.0b013e3181d66c55. (PMID: 20216482)