Hepatoblastoma: Comprehensive Review With Recent Updates.

Area of Science:

• Pediatric oncology
• Hepatobiliary pathology
• Molecular diagnostics

Background:

• Hepatoblastoma (HB) is the most common primary malignant liver tumor in children.
• HB exhibits significant histologic heterogeneity, classified into epithelial and mixed epithelial-mesenchymal subtypes.
• Accurate classification and risk stratification are essential for effective treatment of pediatric liver tumors.

Purpose of the Study:

• To review recent advancements in the histologic classification of Hepatoblastoma.
• To summarize updates in molecular signatures, staging, and risk stratification for HB.
• To highlight the role of international collaboration in standardizing the study and treatment of pediatric liver tumors.

Main Methods:

• Review of current literature on Hepatoblastoma classification and management.
• Analysis of data from the Children's Hepatic tumors International Collaboration (CHETIC).
• Integration of clinical, histologic, and molecular features for risk stratification.

Main Results:

• Distinguishing malignant rhabdoid tumors from small cell undifferentiated HB using INI-1 staining.
• Pure fetal HB can be cured by complete resection alone.
• The CHETIC risk stratification algorithm incorporates age, AFP, metastasis, and pretreatment extent of disease.

Conclusions:

• Refined histologic classification and molecular profiling are improving HB diagnosis and treatment.
• Tailored risk stratification strategies aim to optimize efficacy and reduce treatment toxicity.
• Ongoing international collaboration is vital for advancing the understanding and management of rare pediatric liver tumors.