S5971 Hepatic and Biliary Manifestations of Metastatic Neuroblastoma in Pediatrics

Introduction: Neuroblastomas (NB), considered the most common extracranial solid tumor in pediatrics, possess marked clinical heterogeneity and diverse anatomical site involvement including the liver and biliary tract. Such variable presentation requires vigilant diagnosis and management, particularly in infants wherein complications can arise from the mass effect of metastatic NB, observed in the 2 presented pediatric cases. Case Description/Methods: A 3-month-old female born late preterm with cardiac septal defects, proboscis-type umbilical hernia, and abnormal genetic array was admitted for abdominal distension and poor oral intake. Exam showed markedly distended abdomen with prominent vasculature and firm to palpation with reducible umbilical hernia. Abdominal computed tomography revealed severe hepatomegaly with heterogeneous enhancement and multiple low-attenuation areas. Stage MS NB was confirmed on liver biopsy, and chemotherapy was emergently pursued. Her initial course was complicated by synthetic dysfunction, mild ascites, tumor lysis syndrome, and respiratory compromise. Nonetheless, she completed her therapy with improved abdominal distension, stable liver condition, and repair of umbilical hernia. An 11-month-old term male with left hydronephrosis was admitted for a week of new onset jaundice with pale grey stools. His abdominal exam was unrevealing, and he appeared well-nourished despite jaundice and scleral icterus. With intra- and extra-hepatic biliary ductal dilatation on abdominal ultrasound, magnetic resonance cholangiopancreatography showed aggressive, partially necrotic soft tissue mass along the mesenteric root with mass effect and narrowing of the intrahepatic IVC. Retroperitoneal mass biopsy confirmed stage M NB. Blood studies on admission showed intact synthetic function but transaminitis with direct and indirect hyperbilirubinemia. A week later, his cholestasis worsened, prompting decompression with internal-external biliary catheter. Jaundice has since resolved, and he remains on his chemotherapy course. Discussion: The 2 pediatric cases demonstrate hepatic and biliary complications of metastatic NB, the former at risk of abdominal compartment syndrome and the latter with rising hyperbilirubinemia and encroachment onto nearby vasculature. While obstructive jaundice is a rare event compared to hepatomegaly and abdominal distension in NB, awareness of the wide range of presentations and multidisciplinary efforts are critical for the timely diagnosis and management of these infants.