Outcome in congenital hepatoblastoma compared with previously reported data.

Abstract

10037 Background: Congenital hepatoblastoma, defined as diagnosis in the first month of life (Ammann RA, Plaschkes JLeibundgut K. Congenital hepatoblastoma: A distinct entity? Med Pediatr Oncol 1999:32:466-468.), has been reported to have a poor prognosis; however, a comprehensive evaluation of this entity is lacking. Methods: We performed a retrospective review including three patients from the senior authors’ personal series and 23 cases identified in the databases of several multicenter group studies (INT-0098, P9645, 881, P9346, HB 89, HB94 and HB 99). We then compared this series with the data of all cases of congenital hepatoblastoma previously published in the literature. Results: The overall 2-year survival in our case series was 86% (18/21) with a median followup of 85.5 months (range 44 to 230 months). Presentation and treatment were not substantially different from hepatoblastoma cohorts unselected for age. The infants in our study exhibited a comparable survival rate to the reported disease free survival for a similar cohort of hepatoblastoma patients unselected for age between 1986 and 2002 (82.5%) [Tiao GM, Bobey N, Allen S, et al. The current management of hepatoblastoma: A combination of chemotherapy, conventional resection, and liver transplantation. J Pediatr 2005:146:204-211.]. The overall 2-year survival rate of cases previously reported in the literature was 0% (0/11) for patients reported before 1992, and 47% (8/17) for those reported after 1992. The improved survival of our current series of patients, collected from the past 20 years of German and American multicenter trials and personal series, suggests that the outcome of HB at this young age is much better than has been historically reported. Conclusions: Congenital hepatoblastoma does not appear to confer a worse prognosis. The overall survival in our series does not appear to be worse than in older children; more rigorous analysis should be conducted in future multicenter trials. It is possible that congenital HB should be treated like all other patients with hepatoblastoma provided that the child is stable enough to proceed with surgery and chemotherapy and complete staging is performed.