Hepatoblastoma in Children

What is Hepatoblastoma?

Hepatoblastoma is a tumor in children arising from embryonic or primitive cells. >65% of primary liver malignancies are hepatoblastoma. It arises from primitive hepatic precursors. Age: 95% under 5 years of age.

Risk Factors of Hepatoblastoma

Patients with Familial Adenomatous Polyposis: Gardner's syndrome, beta-catenin pathway mutations

Beckwith - Wiedemann syndrome is associated with Wilm's tumor and hemihypertrophy. Abnormal insulin-like growth factor II levels with this syndrome increase the chances of Hepatoblastoma, Aicardi Syndrome, Trisomy 18, pregnancy, and low birth weight

Clinical Features of Hepatoblastoma

The most common clinical manifestation is asymptomatic abdominal mass. Right-sided lobe >> left-sided lobe. It is a unifocal tumor. Later, constitutional symptoms: weight loss, loss of appetite, fever, malaise, and night sweats. Metastasis: regional lymph nodes > pulmonary. Spreads to hepatic veins and may spread to the inferior vena cava. Portends an unresectable tumor.

Also read: Rhabdomyosarcoma in Children

Diagnosis of Hepatoblastoma

• In blood investigations: a hallmark tumor marker called a-fetoprotein, high levels.
• Anemia and thrombocytosis (30%).
• Imaging: Ultrasonography—solitary, well-defined echogenic mass.
• CT scans and MRIs for spread or tumor delineation.
• A biopsy is done for confirmation.

Histological Subtypes

Epithelial

• Fetal: Best prognosis
• Embryonal
• Small cell undifferentiated: Worst prognosis

Mixed (epithelial + mesenchymal): TEXT

• Small cell form: may also have normal AFP levels
• Mixed form has Intermediate prognosis and they show calcification

Also read: What's New In Paediatric Oncology

Treatment of Hepatoblastoma

About 30% of cases of Hepatoblastoma are found to be surgically resectable. If they are surgically resectable, surgery is followed by post-operated chemotherapy. A large part of the liver has to be removed. Drugs in chemotherapy are a combination of cisplatin with vincristine with or without 5-FU/doxorubicin. The effective dose to kill tumor cells exceeds the normal levels in children. In 70% of cases, they are not surgically resectable at presentation. Two cycles of chemotherapy to reduce the tumor size and then surgery are performed. Post-operated chemotherapy is done. Fail/recurrence: liver transplant is preferred.

Other Hepatic Malignancies in Children

Hepatocellular carcinoma

Secondary or metastasis to the liver, commonly arising from Wilms tumor, lymphoma, or neuroblastoma.

Hepatocellular Carcinoma

• Bimodal
  • Peak (small) → children
  • Peak (large) → adults
• · Risk factor
  • Chronic HBV & HCV
  • Cirrhosis (common in Adults & Rare in Children)
  • Chronic liver disease: α-AT deficiency, Tyrosinemia, Galactosemia, GSD type IV 1
  • Alagille syndrome
  • Exposure to aflatoxins
• Two types of hepatocellular carcinoma
  • Classical
  • Fibrolamellar
• Fibrolamellar Carcinoma:
  • More in Adolescents
  • Not associated with Cirrhosis
  • AFP is normal
  • Associated with a unique translocation: DNAJB1-PRKACA

Treatment for Hepatocellular Carcinoma

The therapy is surgery followed by chemotherapy. In large tumors, chemotherapy is followed by surgery and chemotherapy again. Radiotherapy limited. Liver transplantation can be done if a suitable donor is available.

Also read: Langerhans Cell Histiocytosis in Children

Important Points to Remember

• Hepatoblastoma arises from primitive hepatic precursors. Age: 95% under 5 years of age.
• The most common clinical manifestation is Asymptomatic abdominal mass
• About 30% of cases of Hepatoblastoma are found to be surgically resectable
• Drugs in chemotherapy are a combination of cisplatin with vincristine with or without 5-FU/doxorubicin. The effective dose to kill tumor cells exceeds the normal levels in children.
• Beckwith - Wiedemann syndrome is associated with Wilm's tumor and hemihypertrophy.

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