[Primary liver tumors in children: analysis of 62 consecutive cases]

Aiming at assessing the impact of the improvements introduced in the treatment of primitive liver tumors of children, we reviewed our experience with these tumors since 1980. Between January 1980 and June 1999 we treated 62 children with primitive liver tumors: 35 hepatoblastomas (HB), 5 hepatocarcinomas (HC), 4 sarcomas, 2 malignant mesenchymomas, 11 hemangioendotheliomas (HE), 3 mesenchymal hamartomas, 1 adenoma and 1 focal nodular hyperplasia. Thirty five were boys and 27 girls. The age at diagnosis was 34.5 +/- 43.1 months (mean +/- standard deviation). Eleven patients had HE and 4 of them (36.3%) died due to haemodynamic (n = 3) or other tumor-related causes. All the remaining patients with benign tumors survive. Among the HB patients, 3 had stage I, II stage II, 8 stage III and 13 stage IV tumors (with lung metastases at diagnosis in 4). For survival analysis we divided patients into two groups according to their treatment before (Group 1, n = 14) or after 1991 (Group 2, n = 21). Five-year actuarial survival was 49% for Group 1 and 78% for Group 2 (p < 0.05). We performed liver transplantation in 5 patients with 3 long-term survivals. Three children with widespread HC died whereas 2 with localized tumors had liver transplantation and survive. In conclusion, the treatment of primitive liver tumors in children requires collaborative protocols, concentration of patients in institutions capable of offering high-standard liver surgery and transplantation when the tumor is localized to the liver and irresectable.