From the Guidelines
The prognosis for hepatoblastoma in a 13-month-old child is generally favorable, with overall survival rates of 70-80% when appropriate treatment is provided, as evidenced by the Children’s Oncology Group protocol for hepatoblastoma (COG-AHEP0731) 1. The outlook depends significantly on several factors including tumor stage, histological subtype, complete surgical resection possibility, and response to chemotherapy.
- Key factors influencing prognosis include:
- Tumor stage, with localized disease (PRETEXT stages I and II) having better outcomes
- Histological subtype
- Possibility of complete surgical resection
- Response to chemotherapy For localized disease (PRETEXT stages I and II), survival rates can exceed 90% with complete surgical resection and adjuvant chemotherapy, as suggested by the use of the PRETEXT system to gauge extent of disease at diagnosis and triage patients for early referral to specialized programs 1.
- Standard treatment typically involves:
- Surgical resection when possible
- Cisplatin-based chemotherapy regimens
- Monitoring with alpha-fetoprotein (AFP) levels as an important tumor marker for treatment response and detecting recurrence Children diagnosed at this young age often have better outcomes than older children, as hepatoblastoma is most common in children under 3 years, and early diagnosis and treatment at specialized pediatric cancer centers with multidisciplinary teams experienced in liver tumors provide the best chance for favorable outcomes. Regular follow-up with imaging studies and AFP monitoring will be necessary for several years after treatment completion, especially for patients with pulmonary metastases (PM) at diagnosis, who can have similar recurrence-free survival rates following liver transplantation (LT) if PM are no longer seen by computerized tomography (CT) or residual PM are completely resected and tumor-free margins are identified 1.
From the Research
Hepatoblastoma Prognosis in 13-Month-Old
Overview of Hepatoblastoma
- Hepatoblastoma (HB) is the most common primary liver tumor in children, usually diagnosed during the first 3 years of life 2.
- The pretreatment extent of disease (PRETEXT) staging system is a consensus classification for HB 2.
Treatment Approaches
- Surgical resection is the foundation of therapy in HB, and patients with resectable tumors have an event-free survival (EFS) of 80-90% 3.
- Chemotherapy regimens, such as cisplatin, 5-fluorouracil, and vincristine, can cure patients with standard-risk HB 3, 4.
- For patients with intermediate-risk HB, a combined cisplatin and doxorubicin (PLADO) regimen followed by definitive surgery can result in satisfactory outcomes, with 3-year progression-free and overall survivals of 78.7% and 87.9%, respectively 5.
Prognosis
- Patients with metastatic disease have a poorer prognosis, with EFS ranging from 20-50% 3.
- High-risk patients may benefit from dose intensification of cisplatin and doxorubicin, as well as the use of new agents and liver transplantation 3, 6.
- The outcome for patients with HB can be improved with a better understanding of the biologic and pathologic factors, and the development of more logical risk-based treatments 3.
Risk Stratification
- The Children's Hepatic Tumors International Collaboration (CHIC) has developed a global approach to risk stratification of pediatric HB for use in future global clinical trials 2, 4.
- Risk stratification is crucial in determining the appropriate treatment approach for patients with HB, and can help improve outcomes for patients with high-risk disease 4, 5.