Intracholecystic papillary neoplasm in a pediatric patient: a case report
Eman Alhajj1, Fatima Mhd Rida Hajij2, Mohammad Rashed Al Abdalla1
1Department of General Surgery, Damascus Hospital, Damascus, Syrian Arab Republic.
Insights
Intracholecystic papillary neoplasm (ICPN), a rare biliary tumor, was diagnosed in a pediatric patient. This case highlights diagnostic challenges and the aggressive nature of ICPN in younger individuals.
Area of Science:
- Hepatology
- Pediatric Oncology
- Gastroenterology
Background:
- Intracholecystic papillary neoplasm (ICPN) is a rare premalignant biliary tract tumor, predominantly seen in adults.
- Pediatric cases of ICPN are exceptionally rare, presenting unique diagnostic and clinical challenges.
- The radiological features of ICPN can mimic other biliary malignancies, complicating diagnosis.
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.
