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Hepatoblastoma throughout SIOPEL trials - clinical lessons learnt
1On behalf of SIOPEL group - from Department of Surgery and Urology for Children and Adolescents, Medical University of Gdansk, Poland. pczaud@gumed.edu.pl
Frontiers in Bioscience (Elite Edition)
|December 29, 2011
Summary
Preoperative chemotherapy, including cisplatin-based regimens, significantly improves survival for children with hepatoblastoma (a common childhood liver tumor). Risk stratification using the PRETEXT system guides treatment strategies for better outcomes.
Area of Science:
- Pediatric Oncology
- Hepatobiliary Malignancies
Background:
- Hepatoblastoma is the most common malignant liver tumor in children.
- Preoperative chemotherapy is a cornerstone of treatment, necessitating staging systems like PRETEXT.
Purpose of the Study:
- To evaluate the efficacy of preoperative chemotherapy regimens in hepatoblastoma.
- To assess the impact of risk stratification (standard vs. high risk) on treatment outcomes.
Main Methods:
- The International Childhood Liver Tumors Strategy Group (SIOPEL) conducted multiple studies (SIOPEL 1, 2, and 3).
- Chemotherapy regimens included cisplatin and doxorubicin (PLADO) and cisplatin monotherapy (CDDP).
- Treatment strategies were tailored based on tumor stage (PRETEXT) and risk category (SR/HR).
Main Results:
- SIOPEL 1 demonstrated 5-year survival rates of 75% with PLADO chemotherapy.
- SIOPEL 2 showed improved survival for standard-risk (91% overall survival) versus high-risk (53% overall survival) hepatoblastoma.
- SIOPEL 3 confirmed cisplatin monotherapy is effective and less toxic for standard-risk hepatoblastoma.
Conclusions:
- Cisplatin-based preoperative chemotherapy combined with surgery or transplantation improves survival in pediatric hepatoblastoma.
- The PRETEXT staging system and risk stratification are crucial for optimizing treatment.
- Further research is needed for patients with metastatic disease and low alphafetoprotein levels.
