Abstract
Intrahepatic papillary neoplasm of bile duct configures as a papillary neoplasm with a distinct fibro-vascular stalk layered by variant epithelium pervading intrahepatic bile ducts with cystic dilatation. Neoplasm configures as a premalignant lesion associated with low grade, intermediate grade or high grade intraepithelial neoplasia. Tumour progression is concordant with preliminary inactivation of TP53 and p16, antecedent activating mutations within KRAS and delayed loss of SMAD4/ DPC4 gene. Cogent clinical symptoms arising due to biliary obstruction as hyper-bilirubinaemia or abdominal pain may emerge. Cytological smears are hyper-cellular and comprised of broad, double layered sheets and aggregates of columnar epithelial cells enmeshed within a fine stroma commingled with complex, three dimensional, branching papillary configurations. Morphologically, complex tubulovillous structures or micropapillary articulations appear to distend bile ducts or enlarged, multi-locular cysts are layered by pancreaticobiliary, intestinal, gastric or oncocytic epithelium.
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