Chemotherapeutic approaches for newly diagnosed hepatoblastoma: past, present, and future strategies.
Surgical resection is the foundation of therapy in hepatoblastoma (HB), yet most patients have unresectable tumors at diagnosis. Patients with resectable tumors have event-free survival (EFS) of 80-90% and can be cured with cisplatin, 5-fluorouracil, and vincristine. Patients whose tumors are unresectable but without overt metastases at diagnosis have EFS of 60-70%, and many can be rendered resectable without doxorubicin. Children with metastatic disease have fared poorly with 20-50% EFS, and new approaches for these patients remain desperately needed. Dose intensification of cisplatin and doxorubicin appears beneficial in high-risk patients. Future treatment strategies, which may be useful, include increasing intensity and/or duration of therapy, developing a maintenance regimen (oral irinotecan), using liver transplantation more often for patients to undergo complete resection, and identifying and incorporating novel agents. A better understanding of the biologic and pathologic factors is critical for predicting tumor behavior and developing more logical risk-based treatments.
Department of Pediatric Hematology/Oncology, Sacred Heart Children's Hospital, Spokane, Washington 99204, USA. angela.trobaugh@providence.org
Angela D
AD
Trobaugh-Lotrario
Howard M
HM
Katzenstein
D000293:Adolescent; D000971:Antineoplastic Combined Chemotherapy Protocols / Q000627:therapeutic use*; D002166:Camptothecin / Q000031:analogs & derivatives / Q000627:therapeutic use; D002648:Child; D002675:Child, Preschool; D002945:Cisplatin / Q000627:therapeutic use; D018572:Disease-Free Survival; D004317:Doxorubicin / Q000627:therapeutic use; D005260:Female; D005472:Fluorouracil / Q000627:therapeutic use; D018197:Hepatoblastoma / Q000188:drug therapy* / Q000401:mortality / Q000473:pathology*; D006801:Humans; D007223:Infant; D000077146:Irinotecan; D008113:Liver Neoplasms / Q000188:drug therapy* / Q000401:mortality / Q000473:pathology*; D008297:Male; D009362:Neoplasm Metastasis; D012307:Risk Factors; D015996:Survival Rate; D014184:Transplantation, Homologous
D000077146:Irinotecan; D004317:Doxorubicin; D002945:Cisplatin; D005472:Fluorouracil; D002166:Camptothecin