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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)
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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.

Keywords

cholangiocarcinoma - chemotherapy - liver transplantation - living donor liver transplantation

Schlüsselwörter

Cholangiokarzinom - Chemotherapie - Lebertransplantation - Leberlebendspende


Publikationsverlauf

Eingereicht: 03. September 2023

Angenommen nach Revision: 03. Dezember 2023

Artikel online veröffentlicht:
09. Januar 2024

© 2024. Thieme. All rights reserved.

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Rüdigerstraße 14, 70469 Stuttgart, Germany

 

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