Journal of Pediatric Surgery Case Reports · Published 2026-06-05 · DOI 10.1016/j.epsc.2026.103279
Lielle M. Elisha, Nicholas J. Iglesias, Rebecca A. Saberi, Alan S. Livingstone
Introduction: Neuroendocrine tumors (NETs) of the extrahepatic biliary tree are exceedingly rare in pediatric patients, comprising fewer than 0.2% of all gastrointestinal neuroendocrine neoplasms, and pose a significant diagnostic challenge as their nonspecific obstructive biliary symptoms frequently mimic more common hepatobiliary diseases, leading to delayed diagnosis. Case presentation: A previously healthy 13-year-old male presented with non-specific abdominal pain, hyperbilirubinemia (total bilirubin 1.6 mg/gL), transaminitis (alanine aminotransferase [ALT] 671 U/L, aspartate aminotransferase [AST] 829 U/L), and markedly elevated amylase (3896 U/L). Magnetic resonance cholangiopancreatography (MRCP) demonstrated a T2-hypointense, non-enhancing ovoid lesion within the common hepatic duct with associated proximal biliary dilation. Endoscopic retrograde cholangiopancreatography (ERCP) with cholangioscopy subsequently identified a persistent polypoid intrabiliary lesion, and targeted biopsy confirmed a well-differentiated Grade 2 NET, prompting definitive oncologic resection with Roux-en-Y hepaticojejunostomy reconstruction. Final pathology demonstrated a 1.5 cm well-differentiated Grade 2 NET of the common hepatic duct with negative margins and no nodal involvement (0/2). The postoperative course was uncomplicated and the patient remained clinically well and disease-free at 8-month follow-up. Conclusion: Neuroendocrine tumors of the biliary tree, although rare, should be considered in the differential diagnosis of adolescents presenting with unexplained cholestasis or indeterminate biliary lesions, particularly when initial diagnostic evaluation is inconclusive.
Abstract from DOAJ. Public domain (CC0 1.0).
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Elisha, L., Iglesias, N., Saberi, R., et al. (2026). Well-differentiated Grade-2 neuroendocrine tumor of the common hepatic duct in a teenager: A case report. Journal of Pediatric Surgery Case Reports. https://doi.org/10.1016/j.epsc.2026.103279