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Combined hepatocellular carcinoma-cholangiocarcinoma in a patient with Fontan-associated liver disease
  1. Jonathan Jou1,
  2. Jennifer Li2 and
  3. Fady M Kaldas2
  1. 1Department of Surgery, University of Illinois College of Medicine, Chicago, Illinois, USA
  2. 2Department of Surgery, University of California Los Angeles, Los Angeles, California, USA
  1. Correspondence to Dr Fady M Kaldas; FKaldas{at}


We present a rare case of combined hepatocellular carcinoma-cholangiocarcinoma in a woman with a history of univentricular congenital heart disease requiring multiple corrective operations including Fontan procedure. During workup for elevated alpha fetal protein, a right hepatic lobe lesion was identified with biopsy showing poorly differentiated hepatocellular carcinoma. She underwent successful segment 5 liver resection. Final pathology demonstrated combined hepatocellular carcinoma-cholangiocarcinoma. She was treated with gemcitabine/oxaliplatin adjuvant chemotherapy and had no evidence of recurrent disease at her 12-month follow-up. To our knowledge, this is the first case reported in of successful treatment of this rare malignancy in the setting of Fontan-associated liver disease and highlights the importance of a robust screening protocol in this patient population. Semiannual screening for the development of primary liver malignancy should start by 10 years post-Fontan and continue until heart–liver transplantation may be performed. It is important to note that cirrhosis is not a pre-requisite for the development of hepatocellular carcinoma or cholangiocarcinoma in these patients.

  • Gastrointestinal surgery
  • Cardiothoracic surgery
  • Transplantation
  • Surgical oncology
  • Hepatic cancer

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  • Contributors JJ, JL and FMK contributed equally to the drafting of this manuscript.

  • Funding The authors have not declared a specific grant for this research from any funding agency in the public, commercial or not-for-profit sectors.

  • Case reports provide a valuable learning resource for the scientific community and can indicate areas of interest for future research. They should not be used in isolation to guide treatment choices or public health policy.

  • Competing interests None declared.

  • Provenance and peer review Not commissioned; externally peer reviewed.