Liver transplantation for refractory portal hypertension syndrome due to intrahepatic arterioportal shunts after treatment of primary hepatic choriocarcinoma: A case report

Choriocarcinoma is a malignant germ cell tumor that predominantly affects women of reproductive age and is considered highly curable with chemotherapy. Primary hepatic choriocarcinoma (PHC) is exceptionally rare and occurs without evidence of a gestational trophoblastic origin. We present an adult liver transplantation (LT) for intrahepatic arterioportal shunts that developed after treatment of PHC. A 34-year-old woman presented with hepatosplenomegaly and upper abdominal pain 4 months after an uncomplicated pregnancy. Imaging revealed multiple large partially vascularized liver lesions without extrahepatic disease. A computed tomography-guided biopsy confirmed PHC. Initial serum human chorionic gonadotropin level was markedly elevated at 22 936 mlU/mL. The patient underwent 4 cycles of curative-intent chemotherapy. Despite tumor regression and human chorionic gonadotropin normalization, she developed multiple intrahepatic shunts, resulting in pronounced portal hypertension syndrome. Medical therapy and endovascular coiling were unsuccessful. Transjugular intrahepatic portosystemic shunt was deemed unsuitable, as it risked transitioning the existing shunts into high-flow arteriovenous connections. Persistent portal hypertension syndrome and concern for progression of radiologically stable PHC led to LT. The patient is alive without early recurrence of disease at 9-month follow-up. This is a novel case of LT in a patient with PHC, providing curative therapy for both intrahepatic arterioportal shunts and potential residual tumor.