1. Combined hepatocellular carcinoma-cholangiocarcinoma in a patient with Fontan-associated liver disease
- Author
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Jonathan Jou, Jennifer Li, and Fady M Kaldas
- Subjects
Cholangiocarcinoma ,Carcinoma, Hepatocellular ,Postoperative Complications ,Bile Ducts, Intrahepatic ,Bile Duct Neoplasms ,Liver Neoplasms ,Humans ,Female ,General Medicine - Abstract
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.
- Published
- 2024