Intracholecystic papillary neoplasm in a pediatric patient: a case report.

Introduction: Intracholecystic papillary neoplasm (ICPN) is a rare premalignant tumor of the biliary tract, almost exclusively reported in adults. Case Presentation: A 14-year-old female with a history of cholelithiasis presented with jaundice, right upper quadrant pain, and weight loss. Imaging revealed a massively dilated common bile duct containing a tissue mass. Endoscopic retrograde cholangiopancreatography (ERCP) with biopsy was pivotal, confirming an intraductal papillary neoplasm. Due to refractory obstruction and intraoperative discovery of duodenal invasion, the surgical plan was altered to a pancreaticoduodenectomy (Whipple procedure). Histopathology confirmed ICPN with moderate dysplasia. The patient was discharged but succumbed to massive gastrointestinal hemorrhage 1-month postoperatively. Clinical Discussion: This case expands the known demographic for ICPN to include pediatric patients. The diagnostic challenge is highlighted, as ICPN's radiological features often mimic other biliary malignancies, a difficulty compounded in this case by the unavailability of magnetic resonance cholangiopancreatography. ERCP with biopsy was essential for correct preoperative diagnosis. Despite successful macroscopic resection, the tumor exhibited aggressive local invasion, and the patient suffered a fatal complication, underscoring the high morbidity of major hepatobiliary surgery. Conclusion: This case underscores the exceptional rarity of ICPN in the pediatric population and highlights significant diagnostic challenges. The initial misdiagnosis, partly due to limitations in obtaining advanced imaging, emphasizes that ICPN can mimic other biliary malignancies. A high index of suspicion and the utilization of ERCP with biopsy are crucial for preoperative diagnosis. This report expands the known demographic for this disease and illustrates its potential for aggressive clinical behavior.