Liver Transplantation for Refractory Portal Hypertension Syndrome due to Intrahepatic Arterioportal Shunts Following Treatment of Primary Hepatic Choriocarcinoma: A Case Report

Abstract Choriocarcinoma is a malignant germ cell tumor that predominantly affects women of reproductive age, 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 (AP) shunts that developed following treatment of PHC. A 34-year-old female presented with hepatosplenomegaly and upper abdominal pain four months after an uncomplicated pregnancy. Imaging revealed multiple large partially vascularized liver lesions, without extrahepatic disease. A CT-guided biopsy confirmed PHC. Initial serum human chorionic gonadotropin (hCG) was markedly elevated at 22,936 mlU/mL. The patient underwent 4 cycles of curative-intent chemotherapy. Despite tumor regression and hCG normalization, she developed multiple intrahepatic shunts, resulting in pronounced portal hypertension syndrome (PHS). Medical therapy and endovascular coiling were unsuccessful. Transjugular intrahepatic portosystemic shunt (TIPS) was deemed unsuitable, as it risked transitioning the existing shunts into high-flow arteriovenous connections. Persistent PHS and concern for progression of radiologically stable PHC led to LT. The patient is alive without early recurrence of disease at nine-month follow-up. This is a novel LT in a patient with PHC, providing curative therapy for both intrahepatic AP shunts and potential residual tumor.