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Identification and Successful Treatment of Intrahepatic Cholangiocarcinoma After Fontan Procedure
Matthew W Minick1, Ashley J Limkemann2, Sean G Kelly3
1The Ohio State University College of Medicine, Columbus, Ohio, USA.
Background:
Fontan-associated liver disease (FALD) increases the risk of malignant liver complications in patients with Fontan circulation.
Case Summary:
We present a case of intrahepatic cholangiocarcinoma in a patient with Fontan circulation, diagnosed using targeted biopsy for a hepatic lesion found during heart transplant evaluation. The patient underwent successful tumor resection with adjuvant chemotherapy and has been recurrence-free for 2 years.
Discussion:
Although hepatocellular carcinoma (HCC) is more common in patients with FALD, this is the fifth published case of intrahepatic cholangiocarcinoma in a patient with Fontan circulation, with the longest reported follow-up after successful treatment. Targeted liver biopsy was integral in the diagnosis to provide appropriate treatment.
Take-Home Messages:
Patients with FALD are at risk for both HCC and intrahepatic cholangiocarcinoma. A low threshold for targeted biopsy is important for evaluation of new liver lesions in patients with FALD, even if the most likely diagnosis is HCC.
