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We present a rare cause of jaundice in a young adult female patient with no medical history. MRCP revealed a fusiform dilatation of the common bile duct (CBD) with moderate intrahepatic biliary dilatation, as well as multiple renal cysts. Further evaluation with endoscopic ultrasound revealed multiple septations and stasis within the CBD. The patient was treated surgically with resection of the CBD cyst and hepaticojejunostomy. Histopathological assessment revealed a surprising diagnosis of mucinous cystic neoplasm arising from the cystic duct, confirmed by the presence of ovarian-like stroma (pathognomonic feature). Mucinous cystic neoplasm arising from the cystic duct is extremely rare, with only three other case reports identified in the literature.