Management of hepatic hilar occupied lesions in children: A 23-year single-institution case series

Hepatic hilar occupied lesions (HHOLs) are rare in children with diverse etiologies. This study aimed to present a single-institution case series, review the spectrum of pathologies, and propose a management algorithm. We conducted a retrospective review of pediatric patients (0–18 years) with HHOLs treated at our institution between January 2000 and June 2023. Data on presentation, imaging, laboratory findings, treatment, and outcomes were analyzed. A total of 11 patients were included. Pathologies included biliary rhabdomyosarcoma (RMS, n = 6), biliary yolk sac tumor (YST, n = 1), biliary plexiform schwannoma ( n = 1), biliary cryptococcal infection ( n = 2), and Ewing's sarcoma ( n = 1). The most common presentation was jaundice (7/11). Laboratory findings were variable: alpha-fetoprotein (AFP) was markedly elevated in YST, peripheral blood eosinophils and CA19–9 were significantly increased in cryptococcal infection, while RMS had no specific tumor markers. Imaging (Ultrasound, CT, MRI) was essential for delineating lesions. Management was tailored to pathology: surgical resection for neoplasms (7/11) and antifungal therapy for infections (2/11), with endoscopic or percutaneous biliary drainage used for obstruction. Over a mean follow-up of 9.7 years, one RMS patient died of relapse; all others were alive and event-free. The etiology of pediatric HHOLs is complex, spanning malignant tumors, benign tumors, and infections. Biopsy for pathological or microbiological diagnosis is crucial. Management must be etiology-specific, with surgery reserved for neoplasms and avoided in infections. An algorithm integrating clinical, laboratory, and imaging findings can guide diagnosis and treatment, yielding an encouraging overall prognosis.