Summary: | Our patient is 15-year-old previously healthy female with recent episodes of postprandial right upper quadrant pain now presenting with symptomatic cholelithiasis and suspected choledocholithiasis. The patient underwent an endoscopic retrograde cholangiopancreatography (ERCP) confirming choledocholithiasis and then eventual routine, uncomplicated laparoscopic cholecystectomy. Pathological examination of gallbladder revealed cholelithiasis as well as a noninvasive complex tubular-intracholecystic papillary-tubular neoplasm (ICPN). Expert and literature review of this diagnosis revealed that this lesion is an uncommon, premalignant neoplasm similar to intraductal papillary neoplasms (IPNs) in the bile ducts as well as intraductal papillary mucinous neoplasms (IPMNs) in the pancreas. Our case is the first ICPN reported in a pediatric patient, and they are almost always diagnosed upon pathological evaluation. The features of our patient's lesion were supportive of a benign etiology with a good prognosis, but certain characteristics such as architectural pattern, rate of dysplasia, and cell lineage predict invasion and subsequent management strategies.
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