(CR29) Case Series of Cholangiocarcinoma – A Rare Liver Tumour in Children

 Introduction:

Cholangiocarcinoma (CC) is most commonly diagnosed in the late eighth decade of life and  exceedingly rare before adulthood. Paediatric cholangiocarcinoma is extremely rare with poor  prognosis. The overall incidence rate is 0.0036/100,000 as compared to 1.67/100,000 in adult. We  reported two rare cases of Cholangiocarcinoma in children from our hospital.

Case Reports : 

The first case was a 12 years old girl, presented with progressive abdominal distension and early satiety for 3 months associated with loss of weight and appetite. The liver function test was normal, however the AFP slightly raised and Ca 19-9 was markedly raised. Imaging findings confirmed grossly enlarged liver with heterogenous mass involving both liver lobes. An ultrasound guided biopsy was done and histopathology result was strongly suggestive of Intrahepatic Cholangiocarcinoma. Our second case was an 11 years old girl presented with abdominal discomfort for one week and progressive jaundice. Initial investigation showed raised bilirubin with predominant of direct bilirubin. The Ca 19-9 and AFP were normal. Imaging findings confirmed the presence of heterogeneously enhancing mass at the periportal region resulting in generalized intrahepatic biliary dilatation. Cholangiogram showed filling defect in the common hepatic duct and common bile duct. An ultrasound guided biopsy was performed and histopathology result confirmed Cholangiocarcinoma with focal neuroendocrine differentiation.

Conclusion :

Our two case reports highlight a rare cause of liver tumour in children.