Liver transplantation for refractory portal hypertension syndrome due to intrahepatic arterioportal shunts after treatment of primary hepatic choriocarcinoma: A case report.
Paula Medio, Stephan Singer, Andrea Della Penna et al.
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Case in brief
A 34-year-old woman presented with hepatosplenomegaly and upper abdominal pain four months after an uncomplicated pregnancy. Imaging showed multiple large, partially vascularized liver lesions without extrahepatic disease; biopsy confirmed primary hepatic choriocarcinoma, with serum human chorionic gonadotropin of 22 936 mlU/mL. After four cycles of curative-intent chemotherapy, the tumors regressed and the hormone level normalized, but multiple intrahepatic arterioportal shunts caused pronounced portal hypertension. Medical therapy and endovascular coiling failed, and a transjugular intrahepatic portosystemic shunt was considered unsuitable. Liver transplantation addressed the persistent portal hypertension and concern about residual tumor; she was alive without early recurrence at 9-month follow-up.
What made this case unusual
After chemotherapy response, intrahepatic arterioportal shunts produced refractory portal hypertension, prompting liver transplantation in a patient with primary hepatic choriocarcinoma.
Diagnostic / clinical pearl
Portal hypertension after treatment of hepatic choriocarcinoma may reflect intrahepatic arterioportal shunts even when the tumor has regressed and human chorionic gonadotropin has normalized.