pISSN 2671-8790 eISSN 2671-8804

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Korean J Transplant

Published online August 31, 2021

© The Korean Society for Transplantation

Living donor liver transplantation in an infant patient with progressive familial intrahepatic cholestasis along with hepatocellular carcinoma: a case report

Jung-Man Namgoong1 , Shin Hwang1 , Dae-Yeon Kim1 , Chul-Soo Ahn1 , Hyunhee Kwon1 , Suhyeon Ha1 , Kyung Mo Kim2 , Seak Hee Oh2

1Department of Surgery, Asan Medical Center, University of Ulsan College of Medicine, Seoul, Korea
2Department of Pediatrics, Asan Medical Center, University of Ulsan College of Medicine, Seoul, Korea

Correspondence to: Shin Hwang
Department of Surgery, Asan Medical Center, University of Ulsan College of Medicine, 88 Olympic-ro 43-gil, Songpagu, Seoul 05505, Korea
Tel: +82-2-3010-3930
Fax: +82-2-3010-6701
E-mail: shwang@amc.seoul.kr

Received: April 23, 2021; Revised: June 20, 2021; Accepted: June 21, 2021

This is an Open Access article distributed under the terms of the Creative Commons Attribution Non-Commercial License (http://creativecommons.org/licenses/by-nc/4.0/) which permits unrestricted non-commercial use, distribution, and reproduction in any medium, provided the original work is properly cited.

Abstract

Progressive familial intrahepatic cholestasis (PFIC) is an autosomal recessive inherited disease requiring liver transplantation (LT). Hepatocellular carcinoma (HCC) is very rare in infants. We present a case of living donor LT using a left lateral section graft performed in a 7-month-old female infant diagnosed with PFIC type II and HCC. No mutation on ABCB11 gene was identified. Because of progressive deterioration of liver function, living donor LT with her mother’s left lateral section graft was performed. Pretransplant serum alpha-fetoprotein (AFP) level was increased to 2,740 ng/mL, but HCC was not taken into account because of its rarity. The explant liver showed micronodular liver cirrhosis, multiple infantile hemangiomas and two HCCs of 0.7 cm and 0.3 cm in size. The patient recovered uneventfully from the LT operation. This patient has been regularly followed up with abdomen ultrasonography and AFP measu rement every 6 months. The patient has been continually doing well for 8 years after the LT. In conclusion, LT is currently the only effective treatment for PFIC-associated end-stage liver diseases. HCC can develop at the cirrhotic liver of any cause, thus elevation of HCC tumor markers in pediatric patients is an important clue to perform further investigation before LT.

Keywords: Infant, Hepatocellular carcinoma, Tumor marker, Liver cirrhosis, Carcinogenesis