Liver Tumors

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)

Diagnosis

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

Imaging

• 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

Treatment

• 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