What is the treatment approach for a liver tumor in a child?

Treatment Approach for Liver Tumors in Children

The treatment approach for pediatric liver tumors should be based on tumor type, with hepatoblastoma typically treated with perioperative chemotherapy followed by complete surgical resection, while unresectable tumors should be referred for liver transplantation evaluation at diagnosis or after initial chemotherapy. 1

Diagnosis and Classification

Common Pediatric Liver Tumors

• Malignant tumors:

  • Hepatoblastoma (HB): Most common, typically in younger children (under 3 years)
  • Hepatocellular carcinoma (HCC): More common in older children, often associated with underlying liver disease
  • Rare malignancies: Biliary rhabdomyosarcoma, angiosarcoma, undifferentiated sarcoma 2, 3

• Benign tumors:

  • Infantile hemangioma
  • Mesenchymal hamartoma
  • Focal nodular hyperplasia 2

Initial Evaluation

• Alpha-fetoprotein (AFP) measurement: Typically elevated (>400 ng/dL) in hepatoblastoma
• Imaging studies: CT or MRI with contrast to determine extent of disease
• PRETEXT (Pretreatment Extent of Disease) staging for hepatoblastoma to guide treatment approach 1

Treatment Algorithm for Hepatoblastoma

1. Resectable Tumors

• First-line approach: Perioperative chemotherapy followed by complete surgical resection of all viable tumor 1
• Cisplatin-based chemotherapy regimens are standard (typically 2-4 rounds before assessing resectability)

2. Initially Unresectable Tumors

• Key recommendation: Children with nonmetastatic and otherwise unresectable hepatoblastoma should be referred for liver transplantation evaluation at diagnosis or no later than after 2 rounds of chemotherapy 1
• Tumors likely to be unresectable include:
  • PRETEXT IV (all four liver sections involved)
  • Complex PRETEXT III (multifocal or with venous thrombosis)
  • Centrally located tumors where tumor-free margins are unlikely 1

3. Metastatic Disease

• Pulmonary metastases: Liver transplantation can be considered if:
  • Metastases resolve completely with chemotherapy, or
  • Residual metastases can be completely resected with tumor-free margins 1
• Primary liver transplantation for unresectable HB has 82% 10-year survival vs. 30% for "rescue" transplantation after failed resection 1

Treatment Algorithm for Hepatocellular Carcinoma

1. Resectable Disease

• Primary approach: Surgical resection for single tumors with well-preserved liver function 1
• Unlike adults, 60-70% of pediatric HCC occurs in non-cirrhotic livers 1

2. Unresectable Disease

• Liver transplantation may be considered, though adult Milan criteria may not be directly applicable to children 1
• Treatment must be individualized based on extent of disease 4
• For advanced disease: Consider sorafenib or clinical trials 1

3. Surveillance for High-Risk Children

• Children with predisposing conditions (Beckwith-Wiedemann syndrome, FAP, tyrosinemia) should undergo regular surveillance with ultrasound and AFP measurements 1

Special Considerations

Surgical Approaches

• Complete surgical resection is critical for cure in both HB and HCC
• Laparoscopic liver resection may be considered for tumors in favorable locations 1
• For recurrent tumors after initial cure, treatment approach should consider:
  • Timing of recurrence
  • Remnant liver function
  • Performance status
  • Size, location, and number of recurrent tumors 1

Multidisciplinary Management

• Treatment should be coordinated at a pediatric liver transplant center with expertise in:
  • Pediatric gastroenterology/hepatology
  • Pediatric oncology
  • Pediatric liver transplant surgery
  • Intensive care specialists 1

Common Pitfalls to Avoid

1. Delayed referral: Early referral to a specialized center is critical, especially for potentially unresectable tumors
2. Inappropriate application of adult criteria: Milan criteria for HCC transplantation may not be directly applicable to children
3. Delayed consideration of transplant: "Rescue" transplantation after failed resection has significantly worse outcomes than primary transplantation for unresectable disease
4. Inadequate staging: Complete assessment of metastatic disease is essential before determining treatment approach

The management of pediatric liver tumors requires a specialized multidisciplinary approach at centers with expertise in pediatric liver disease and transplantation to optimize outcomes and survival.