An unusual cause of obstructive jaundice in childhood: Intra-choledochal malignant neuroendocrine tumour.

The extrahepatic biliary apparatus is a rare site for neuroendocrine tumours. A 13-year-old child presented with cholestatic symptoms of jaundice and pruritus with soft hepatomegaly and mild ascites. Magnetic resonance imaging and endoscopic ultrasound revealed a mid-common bile duct mass, and dilated intrahepatic biliary system. An en-bloc resection of the extrahepatic biliary apparatus, showed malignant cells disposed in lobules in a desmoplastic stroma with intramural invasion, staining positive for cytokeratin, chromogranin, synaptophysin and negative for CD56. At 3 months post-resection, whole body positron emission tomography scan was normal with no recurrence at 24 months.