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BACKGROUND: Fibrolamellar hepatocellular carcinoma (FL-HCC) is a rare liver malignancy affecting predominantly young patients, though management can differ between pediatric and adult populations. We evaluated age-related differences in treatment and overall survival (OS). METHODS: Patients with FL-HCC diagnosed from 2004 to 2021 were identified in the National Cancer Database. Demographic, tumor, and treatment characteristics were compared, and OS was assessed using Kaplan-Meier analysis. RESULTS: Among 487 patients, 172 were children (< 18 years) and 315 were adults (≥ 18 years). Children more frequently underwent lymph node removal (80.0% vs 58.6%) and had higher positive-margin rates (15.1% vs 6.7%). Five-year OS was 70.5% in children versus 61.6% in adults. On multivariable analysis, adulthood (HR 1.38, 95% CI 1.00-1.89), metastasis at diagnosis (HR 3.47, 95% CI 2.39-5.02), and positive margins (HR 3.16, 95% CI 2.15-4.64) were associated with increased mortality. CONCLUSIONS: Pediatric patients with FL-HCC had superior OS despite similar disease burden. Positive margins were strongly associated with mortality, supporting efforts to achieve R0 resection.
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Malkawi et al. (2026) studied this question.
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