Pseudotumoral Hilar Lesion Mimicking Cholangiocarcinoma Revealing Primary Sclerosing Cholangitis in a Child

Abstract

Primary sclerosing cholangitis (PSC) is a rare cause of pediatric cholestasis and may exceptionally present as a focal mass-forming lesion. We report the case of a 9-year-old boy presenting with progressive obstructive jaundice in whom magnetic resonance cholangiopancreatography revealed a solid lesion at the hepatic duct confluence, highly suspicious for malignancy. Given persistent biliary obstruction and the inability to reliably exclude cholangiocarcinoma using noninvasive or endoscopic diagnostic methods, surgical exploration was undertaken. Complete excision of the lesion followed by Roux-en-Y hepaticojejunostomy was performed. Histopathological examination demonstrated concentric periductal fibrosis consistent with PSC, associated with established biliary cirrhosis, without evidence of malignancy. The postoperative course was uneventful, with rapid clinical and biochemical improvement. This case highlights a rare pseudotumoral presentation of pediatric PSC and underscores the role of surgery as a definitive diagnostic and therapeutic option when malignancy cannot be excluded.