A review of 218 pediatric cases of hepatocellular carcinoma

Abstract Purpose This study evaluates the incidence trends and clinical outcomes of children with hepatocellular carcinoma (HCC) and assesses factors predictive of patient survival. Methods The Surveillance, Epidemiology, and End Results registry was queried from 1973 to 2009 for all patients between ages 0 and 19 with primary HCC. Demographics, tumor histology, surgical intervention, and patient survival were collected. Results Overall, 218 patients were identified. The annual age-adjusted incidence was 0.05 cases per 100,000 in 2009. Fibrolamellar subtype tumors were exclusive to children > 5 years old and exhibited greater survival compared to non-fibrolamellar subtype (57% vs. 28%, respectively, p = 0.002). Tumor extirpation for patients with resectable disease significantly improved overall survival at 5 years compared to no surgery (60% vs. 0%, respectively, p < 0.0001). Overall 5-, 10- and 20-year survival for the entire cohort was 24%, 23%, and 8%, respectively. Independent prognostic factors of lower mortality according to multivariate analysis were surgical resection (hazard ratio (HR) = 0.18), non-Hispanic ethnicity (HR = 0.52), and local disease at presentation (HR = 0.46). Conclusion Over the past four decades, the incidence of HCC has remained relatively stable. Children of Hispanic ethnicity have high mortality rates. However, HCC resection for curative intent significantly improves outcomes.