Hepatoblastoma (HB) is the most common primary pediatric hepatic malignancy with most cases occurring before the child’s fourth birthday. Like Wilms’ tumor, survival in hepatoblastoma has been improved dramatically with refinement of systemic chemotherapy and improved surgical techniques. A number of multicenter trials will be discussed in this topic that have enhanced our understanding of tumor biology and the effectiveness of the various chemotherapy regimens. With survival improving, current and future research protocols will increasingly focus on risk adapted therapy that maintains excellent survival for low and standard risk patients while reducing toxicity of the therapy. For higher risk patients we continue to pursue new therapies that may improve outcome.
Complete surgical resection of the primary lesion is essential for cure of HB independent of stage and risk strata. Currently, the pretreatment extent of disease (PRETEXT) classification originally developed by the European Liver Tumor Strategy Group (SIOPEL) provides the best framework for planning these resections which vary from segmentectomy to total hepatectomy and orthotopic liver transplantation. The timing of resection is of increasing importance as treatment is based on risk assessment. Upfront resection of PRETEXT I or II lesions, and biopsy followed by chemotherapy and delayed resection for PRETEXT III or IV lesions is now standard in the United States. Delayed resection is also recommended for most tumors with positive PRETEXT Annotation Factors such as macrovascular invasion (V and P), multifocality (F), rupture (R) or contiguous extension into neighboring structure (E). Details of the PRETEXT system and surgical resection guidelines are provided in the Staging and Indications for Surgery modules of this topic.
Other modules focus on pre- and postoperative care, the anatomy of the liver, current methods used for liver resections, transplantation, the current approach to metastatic disease and recommendations for follow up.
Throughout, the topic will focus on achieving optimal outcomes. This goal may be best achieved with protocol based therapy and attempts to minimize the morbidity of both chemotherapy and surgery. Many feel that children with large extensive tumors will achieve the best possible survival results if they are treated at higher volume centers with experience in complex liver resection. Tension exists between referral to such centers and caring for children within their community of origin where family support may be maximized and financial and emotional stresses decreased. Few data have been reported to help guide objective decisions between these competing goals.
Ultimately this topic seeks to aid surgeons as they strive to provide surgical care for children with HB that is effective, timely, safe and patient centered.
see also Hepatectomy for Hepatoblastoma
Content in this topic is referenced in SCORE Malignant Liver Tumors: Hepatoblastoma/Hepatocellular Carcinoma overview
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