The current management of hepatoblastoma: a combination of chemotherapy, conventional resection, and liver transplantation.

OBJECTIVE

To review our experience in the management of children who present with hepatoblastoma.

STUDY DESIGN

Thirty patients treated for hepatoblastoma at a single institution were reviewed.

RESULTS

Ten patients presented with stage I to stage II disease and underwent resection. Seventeen presented with stage III disease; two underwent initial resection of which one required rescue transplantation. The remaining 15 underwent biopsies, which were followed by chemotherapy. Nine patients had a reduction in tumor size and underwent conventional resection. One required rescue transplantation for residual disease. Five patients underwent primary transplantation for unresectable disease. One patient expired during chemotherapy. Three patients presented with stage IV disease and underwent biopsies, which were followed by chemotherapy. One patient responded but required "rescue" transplantation after conventional resection. Seven patients underwent aggressive conventional resection (trisegmentectomy or central liver resection); three had positive surgical margins and underwent transplantation. One developed recurrent disease. Five-year survival was 82.5% +/- 7.1%. There was no operative mortality during surgical therapy. All transplant recipients were tumor free, but one died from lymphoma 7 years post-transplant.

CONCLUSION

Chemotherapy may reduce tumor size, allowing for conventional resection. If aggressive resection is necessary or bi-lobar disease persists, primary transplantation is recommended.