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Published on: September 25, 2013
Primary malignant liver tumors in children
1Department of Pediatric Surgery, All India Institute of Medical Sciences, New Delhi, 110029, India. sandpagr@hotmail.com
Indian Journal of Pediatrics
|March 3, 2012
Summary
Pediatric liver tumors, primarily hepatoblastoma (HB), require multimodal treatment including surgery and chemotherapy. Advances have improved survival for HB, but hepatocellular carcinoma (HCC) outcomes remain poor.
Area of Science:
- Pediatric oncology
- Hepatobiliary surgery
- Medical oncology
Background:
- Liver tumors are rare in children, accounting for 1-4% of pediatric solid tumors, with two-thirds being malignant.
- Hepatoblastoma (HB) is the most common malignant pediatric liver tumor, followed by hepatocellular carcinoma (HCC) and other rare types.
- Accurate diagnosis relies on imaging, elevated alpha-fetoprotein (AFP) levels, and histopathology.
Purpose of the Study:
- To review the current understanding and management strategies for pediatric liver tumors, focusing on hepatoblastoma.
- To discuss staging systems, treatment modalities, and survival outcomes for various pediatric liver tumors.
- To highlight recent advancements and challenges in treating these rare malignancies.
Main Methods:
- Review of diagnostic criteria including radiology, alpha-fetoprotein (AFP) levels, and histology.
- Discussion of the PRETEXT staging system for risk stratification and treatment planning.
- Analysis of treatment modalities: surgery, chemotherapy (cisplatin-based), and liver transplantation.
- Evaluation of survival rates and treatment toxicities based on current literature and clinical trials.
Main Results:
- Complete surgical resection is crucial for survival, with chemotherapy rendering initially unresectable tumors resectable in over 85% of cases.
- Current 3-year overall survival for hepatoblastoma (HB) is 62-70%, with liver transplantation achieving an 85% survival rate for unresectable cases.
- Hepatocellular carcinoma (HCC) in children still has poor outcomes, while rhabdoid tumors and angiosarcomas are chemoresistant; undifferentiated sarcoma and rhabdomyosarcoma show better chemotherapy response.
Conclusions:
- Multimodal treatment strategies, including risk-adapted chemotherapy and surgery, have significantly improved outcomes for hepatoblastoma (HB).
- Liver transplantation offers a viable option for unresectable pediatric liver tumors, achieving high survival rates.
- Continued research is essential to improve treatment efficacy, reduce toxicity, and enhance survival for all pediatric liver tumor types, especially HCC.
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