A case of Fontan-related hepatocellular carcinoma successfully treated with proton beam therapy

  • Yuri Ogasawara
  • Tomomi KogisoEmail author
  • Takaomi Sagawa
  • Kazuhisa Kodama
  • Makiko Taniai
  • Haruko Numajiri
  • Hideyuki Sakurai
  • Katsutoshi Tokushige
Case Report


A 27-year-old woman was admitted to our hospital due to a liver tumor. She had been born late at 41 weeks of gestation and had heterotaxy syndrome, polysplenia, and complete transposition of the great arteries. She underwent percutaneous balloon angioplasty at 5 years of age and the Fontan procedure at 6 years of age. At 25 years of age, computed tomography detected liver congestion. Her alpha-fetoprotein level increased from 13 to 2098 ng/dL (L3 fraction 1.8%) at 27 years of age. Gadolinium ethoxybenzyl diethylenetriamine pentaacetic acid enhanced magnetic resonance imaging showed a 22-mm liver tumor in the second liver segment. The liver tumor was enhanced in the arterial phase and washed-out in the hepatobiliary phase; the patient was, therefore, diagnosed with hepatocellular carcinoma. Radiofrequency ablation and surgery were not indicated due to the proximity of the tumor to the inferior vena cava. Therefore, proton beam therapy was selected as conservative therapy, and a dose of 74 Gray equivalents in 37 fractions was administered at the University of Tsukuba Hospital. There were no severe adverse events and she survived for 38 months after treatment without recurrence.


Fontan procedure Fontan-associated liver disease (FALD) Hepatocellular carcinoma (HCC) Proton beam therapy (PBT) 



We would like to express our gratitude to Professor T. Nakanishi, Professor H. Sugiyama, and Dr. T. Shinohara of the Institutes of Cardiology and Pediatric Surgery for performing the Fontan procedure and follow-up of the patient.

Compliance with ethical standards

Conflict of interest

Professor Katsutoshi Tokushige received research funding from Sumitomo Dainippon Pharma; Astellas Pharma; Eisai; TAIHO Pharmaceutical; Chugai Pharmaceutical; Daiichi Sankyo Pharmaceutical; AbbVie GK; Takeda Pharmaceutical Company; Asahi Kasei Corporation; AJINOMOTO; and Otsuka Pharmaceutical. The other authors have no conflicts of interest.

Human/animal rights

All procedures were performed in accordance with the ethical standards mandated in the 1964 Declaration of Helsinki and its later amendments.

Informed consent

Informed consent was obtained from the patient.


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Copyright information

© Japanese Society of Gastroenterology 2019

Authors and Affiliations

  1. 1.Institute of Gastroenterology, Department of Internal MedicineTokyo Women’s Medical UniversityShinjuku-kuJapan
  2. 2.The Proton Beam Therapy Center of the University of Tsukuba HospitalTsukubaJapan

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