Pediatrics, Baylor College of Medicine.
Pathology, Baylor College of Medicine.
Pediatrics- Oncology, Baylor College of Medicine.
Pathology, Pediatrics- Oncology, Baylor College of Medicine.
Pediatrics- Gastroenterology, Baylor College of Medicine.
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.
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's exact test and Wilcoxon test were employed for sub-group comparison, and survival differences were evaluated by Kaplan-Meier method.
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 condition: cryptogenic cirrhosis/steatosis (9), genetic (7), biliary pathology (4), viral hepatitis (1), other (4). Thirteen of 27 patients with de novo HCC had fibrolamellar HCC. Clinical characteristics were grouped by presence or absence of predisposing conditions: age at diagnosis (7.2 versus 10.2 years, p < 0.05), metastatic disease at presentation (15% versus 44%, p = n.s), and tumor size >4 cm (20% versus 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).
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.