Intrahepatic cholangiocarcinoma developing seven years after resection of congenital biliary dilatation

Abstract

A 13-year-old girl developed intrahepatic cholangiocarcinoma 7 years after laparoscopic extrahepatic bile duct resection and biliary reconstruction to treat Todani's type IV-A congenital biliary dilatation (CBD). During the postoperative period, her serum carbohydrate antigen 19-9 levels were above normal, and the imaging tests showed intrahepatic bile duct dilatation. Then, 7 years after the initial operation, she presented to our hospital with stomachache. Computed tomography revealed an irregular tumor in segment 3 of the liver and swelling of her hepatoduodenal lymph node. She was suspected of having left intrahepatic cholangiocarcinoma with hepatoduodenal lymph node metastases. Therefore, she underwent left hepatectomy and regional lymph node dissection. The histopathological diagnosis after resection was intrahepatic cholangiocarcinoma with poorly differentiated adenocarcinoma. According to the Union for International Cancer Control, 8th edition, the TNM classification of malignant tumors was pT3, pN1, pM0, pStage IVA. Subsequently, 4 months after undergoing left hepatectomy, she had a metastatic recurrence of intrahepatic cholangiocarcinoma. Although the significant association between CBD and bile duct cancer after surgery for CBD is well known for adults, we emphasize that children also have the risk of cancer development.

Keywords

• Congenital biliary dilatation
• Intrahepatic cholangiocarcinoma
• Pediatric surgery