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Key Features

Essentials of Diagnosis

  • Abdominal enlargement and pain, weight loss, anemia

  • Hepatomegaly with or without a definable mass

  • Mass lesion on imaging studies

  • Laparotomy and tissue biopsy

General Considerations

  • Primary neoplasms of the liver represent 0.3–5% of all solid tumors in children

  • Of these, two-thirds are malignant

  • Hepatoblastoma

    • Most common (79% of all pediatric liver cancers)

    • Typically occurs in children ages 6 months to 3 years

    • Male predominance

  • Hepatocellular carcinoma

    • Most commonly occurs between the ages of 10 and 12 years

    • More common in males

Clinical Findings

Symptoms and Signs

  • Noticeable abdominal distention, with or without pain, is the most constant feature

  • Constitutional symptoms (eg, anorexia, weight loss, fatigue, fever, and chills) may be present

  • Jaundice or pruritus may be seen if obstruction of the biliary tree occurs

  • Virilization has been reported as a consequence of gonadotropic activity of tumors

  • Physical examination reveals hepatomegaly with or without a definite tumor mass, usually to the right of the midline

  • Hepatoblastoma

    • Symptomatic abdominal mass present in most children

    • Weight loss, anorexia, abdominal pain, and emesis may occur in more advanced disease

    • Children with Beckwith-Wiedemann syndrome and familial adenomatosis polyposis coli are at increased risk for hepatoblastoma

  • Hepatocellular carcinoma

    • Abdominal distention, pain, anorexia, and weight loss

    • Risk factors: chronic HBV or HCV infection, cirrhosis, glycogen storage disease type I, tyrosinemia, and α1-antitrypsin deficiency

Differential Diagnosis

  • Hematologic and nutritional conditions

  • Hepatitis B and C virus infection

  • α1-Antitrypsin deficiency disease

  • Lipid storage diseases

  • Histiocytosis X

  • Glycogen storage disease

  • Tyrosinemia

  • Congenital hepatic fibrosis

  • Cysts

  • Adenoma

  • Focal nodular hyperplasia

  • Hemangiomas

  • Hepatic abscess (pyogenic or amebic)


Laboratory Findings

  • Normal liver function tests are the rule

  • Anemia frequently occurs, especially in cases of hepatoblastoma

  • α-Fetoprotein levels are typically elevated, especially in hepatoblastoma

  • Estradiol levels are sometimes elevated

  • Tissue diagnosis is best obtained at laparotomy, although ultrasound- or CT-guided needle biopsy of the liver mass can be used


  • Ultrasonography, CT, and MRI are useful for diagnosis, staging, and following tumor response to therapy

  • A scintigraphic study of bone and chest CT are generally part of the preoperative workup to evaluate metastatic disease


  • For tumors that are resectable, an aggressive surgical approach with complete resection of the lesion offers the only chance for cure

  • Individual lung metastases should also be surgically resected

  • Radiotherapy and chemotherapy: hepatoblastomas are generally more responsive than hepatocellular carcinoma

  • Liver transplantation can be an option in hepatoblastoma with unresectable disease limited to the liver, with an 85% 10-year survival

  • For hepatocellular carcinoma, the survival rate is poor due to the typically advanced stage at diagnosis

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