Published online Nov 27, 2019. doi: 10.4240/wjgs.v11.i11.414
Peer-review started: July 16, 2019
First decision: August 2, 2019
Revised: September 7, 2019
Accepted: September 25, 2019
Article in press: September 25, 2019
Published online: November 27, 2019
Processing time: 145 Days and 19.4 Hours
Intrahepatic bile duct adenoma (BDA) is one of the rarest of the rare benign tumors of the liver in the pediatric age group. It arises from the epithelial lining of intrahepatic bile ducts. The overall incidence of BDA is 1.3% of all primary benign liver tumors. Few case reports of this rare tumor occurring in adult population are present in the literature and to date, only one pediatric case has been reported worldwide.
An 18-month-old male child presented with chief complaints of mass per abdomen for 8 mo. Computerized tomography abdomen revealed a well-defined exophytic solid tumor arising from the posteroinferior margin of the right lobe of the liver with heterogenous enhancement and cystic changes, suggesting a suspicion of hepatoblastoma. Non-anatomical liver resection was done and a tumor of 10 cm × 9.5 cm was excised. Histopathology of the specimen was conclusive with the diagnosis of intrahepatic bile duct adenoma, which was further supported by immunohistochemistry panel testing. The post-operative period was uneventful. On follow-up, the child was doing well and had no post-operative complication.
Intrahepatic bile duct adenoma in children is very rare and to date only one case has been reported. This is the second pediatric case of intrahepatic bile duct adenoma in the world. Additionally this is the first ever case of such a large tumor presenting in a child.
Core tip: Intrahepatic bile duct adenoma is a rare tumor arising from bile ductules in the liver. It is mostly seen as an incidental finding in adults but it is almost unheard of in children. Therefore it is one of the rarest tumors in children. Although it is a benign tumor, it has the potential to be malignant according to one study reported in adults. This case highlights the fact that it is the second case of this rare tumor occurring in the pediatric age group in the world. The progression into a large tumor in our case report necessitates the need for more information on this rare tumor.