What is the treatment approach for a child presenting with hepatoblastoma, swelling, and lymphadenopathy?

Treatment Approach for Hepatoblastoma in a Child with Swelling and Lymphadenopathy

The gold standard treatment for hepatoblastoma in a child presenting with swelling and lymphadenopathy consists of perioperative cisplatin-based chemotherapy followed by complete surgical resection of all viable tumor, with early referral for liver transplantation evaluation if the tumor appears unresectable. 1

Initial Assessment and Staging

• PRETEXT Staging System: Essential for determining extent of disease and guiding treatment decisions

  • PRETEXT IV: Disease involving all four liver sections
  • Complex PRETEXT III: Multifocal disease or presence of venous thrombosis
  • Centrally located tumors: May make tumor-free excision unlikely 1

• Metastatic Evaluation:

  • Chest CT to evaluate for pulmonary metastases (most common site)
  • Comprehensive lymph node assessment (given the presentation with lymphadenopathy)
  • Alpha-fetoprotein (AFP) measurement - typically elevated in hepatoblastoma 1, 2

Treatment Algorithm

1. Initial Chemotherapy

• Begin with cisplatin-based chemotherapy (2-4 rounds) to:
  • Reduce tumor size
  • Improve resectability
  • Treat potential metastatic disease 1, 2

2. Surgical Planning Based on Response

• If tumor becomes resectable:

  • Proceed with conventional partial hepatectomy with tumor-free margins
  • Complete surgical resection is crucial for cure 1, 3

• If tumor remains unresectable after chemotherapy:

  • Proceed to liver transplantation evaluation
  • Avoid "heroic" partial resections that risk leaving residual tumor 4

3. Indications for Primary Liver Transplantation

• PRETEXT IV disease (involving all four liver sections)
• Complex PRETEXT III disease (multifocal or with venous thrombosis)
• Centrally located tumors involving main hilar structures or hepatic veins
• Any tumor configuration where complete resection with adequate margins is doubtful 1, 4

4. Management of Pulmonary Metastases and Lymphadenopathy

• Patients with pulmonary metastases can still be considered for liver transplantation if:
  • Metastases disappear completely on chest CT after chemotherapy, OR
  • Residual metastases can be completely resected with tumor-free margins 1
• Lymphadenopathy should be thoroughly evaluated to determine if it represents metastatic disease
• Complete resection of involved lymph nodes should be performed when possible 1, 2

Prognostic Considerations

• Favorable prognostic factors:

  • Complete surgical resection (most important factor)
  • Good response to chemotherapy
  • Absence of metastatic disease 2

• Survival rates:

  • 5-year survival >90% for initially resectable disease
  • 82% 10-year survival for primary liver transplantation for unresectable disease
  • 30% 10-year survival for "rescue" transplantation after recurrence 1, 2

Important Caveats

• Early referral to a specialized pediatric liver transplant center is critical - ideally at diagnosis or no later than after 2 rounds of chemotherapy 1
• Avoid partial hepatectomy in cases where radical resection appears difficult or doubtful, as incomplete resection significantly worsens prognosis 4
• Nearly 25% of children with hepatoblastoma in the US never receive surgical treatment, representing a missed opportunity for potentially curative therapy 5
• Multidisciplinary care involving pediatric oncology, pediatric surgery, transplant surgery, and intensive care specialists optimizes outcomes 1

Follow-up Care

• Regular monitoring of AFP levels to detect recurrence
• Surveillance imaging to monitor for recurrent disease
• Long-term monitoring for late effects of chemotherapy and surgery 2