Predisposing Conditions to Pediatric Hepatocellular Carcinoma and Association With Outcomes: Single-center Experience

• Published in: Journal of pediatric gastroenterology and nutrition Vol. 68; no. 5; pp. 695 - 699
• Main Authors: Cowell, Elizabeth, Patel, Kalyani, Heczey, Andras, Finegold, Milton, Venkatramani, Rajkumar, Wu, Hao, López-Terrada, Dolores, Miloh, Tamir
• Format: Journal Article
• Language: English
• Published: United States by European Society for Pediatric Gastroenterology, Hepatology, and Nutrition and North American Society for Pediatric Gastroenterology 01.05.2019
• ISSN: 0277-2116; 1536-4801
• DOI: 10.1097/MPG.0000000000002285

OBJECTIVES:Hepatocellular carcinoma (HCC) has been linked to chronic viral or metabolic liver disease and other conditions. The characteristics of children with HCC have not been fully elucidated and outcomes in children with predisposing liver disease are not well defined. METHODS:Patients ≤21 years old with HCC managed at our institution and through external consultation between 1996 and 2016 were included. Demographics, clinical history, and pathology were tabulated. Fisher exact test and Wilcoxon test were employed for subgroup comparison, and survival differences were evaluated by Kaplan-Meier method. RESULTS:Sixty-one cases of HCC were identified. Seven of 16 patients (44%) at our institution and 18 of 45 consult patients (40%) had a predisposing conditioncryptogenic cirrhosis/steatosis (9), genetic (7), biliary pathology (4), viral hepatitis (1), and other (4). Thirteen of 27 patients with de novo HCC had fibrolamellar HCC. Clinical characteristics were grouped by presence or absence of predisposing conditionsage at diagnosis (7.2 vs 10.2 years, P < 0.05), metastatic disease at presentation (15% vs 44%, P = n.s), and tumor size >4 cm (20% vs 100%, P < 0.05). In patients treated at our institution, 5 of 7 with predisposing conditions received liver transplant and achieved complete remission, whereas only 3 of 9 patients with de novo HCC received curative surgery and this group had decreased median overall survival (P < 0.05). CONCLUSIONS:The majority of children with HCC did not have predisposing liver or associated disease. These patients were diagnosed later with more advanced stage disease and had significantly decreased overall survival.