Incidental intrahepatic cholangiocarcinoma detected in the explant liver after living-donor liver transplantation for polycystic liver disease.

Living-donor liver transplantation (LDLT) is considered for polycystic liver disease (PLD) with portal hypertension or quality of life deterioration. However, concomitant malignant tumors are extremely rare in PLD. The recipient was a 68-year-old female who had been followed for PLD. She developed progressive jaundice with ascites and lower leg edema. Computed tomography revealed portal hypertension with intrahepatic portal vein stenosis and splenorenal shunt. The liver was replaced by numerous cysts, but no obvious tumorous lesions were identified. Tumor markers were CEA of 7.9 ng/ml and CA19-9 of 420 U/ml. With a MELD score of 12 and Child-Pugh grade C, LDLT was planned. The living liver donor was her 42-year-old daughter, and a right lobe graft was planned for the transplantation with predicted graft-to-recipient weight ratio (GRWR) of 0.88. Portal inflow modulation was performed with proximal splenic artery embolization before LDLT. The operation time was 351 min and the intraoperative blood loss was 2,100 ml. The recipient was discharged on postoperative day 17 without any complications. Although no macroscopic tumor was observed in the explanted liver, histopathological examination revealed adenocarcinoma with papillary proliferation in the cyst wall containing hematoma. Biliary intraepithelial neoplasia (BilIN-1) was also found in the adjacent cysts. The recipient remains well without recurrence with good graft function at 2.5 years after LDLT. We herein report an extremely rare case of incidental intrahepatic cholangiocarcinoma detected in the explanted liver after LDLT for PLD. The possibility of concomitant malignant tumor should be considered even in PLD.