Hepatoblastoma is a type of liver cancer which is seen in young children. Surgical resection is usually required for cure, but not all tumors are removable.

Incidence (How common is Hepatoblastoma?):
Hepatoblastoma occurs in approximately 1 per million children. It is more common in males than females in children younger than 5 years (1.2:1 to 3.6:1), but the incidence is equal in children older than 5. It does, however, occur 4 to 5 times more frequently in white children than African-American children.

Associated Syndromes:
Beckwith-Wiedemann syndrome and Familial Adenomatous Polyposis.

Maternal exposure to metals, paints, and oil products, prematurity with prolonged hospitalization

Clinical Presentation:

  • 35% of patients have bilateral (both sides of the liver) disease
  • 20% have distant metastasis at time of diagnosis
  • Most common site for spread is lung. Other common sites include brain and bone.

Histology (microscopic appearance)

  • 56% epithelial origin
  • 31% pure fetal
  • 19% embryonal
  • 3% macrotrabecular
  • 3% small cell undifferentiated

Epithelial tumors (Fetal or Embryonal) usually have a homogeneous appearance (all the cells look the same).


  • 44% mixed epithelial and mesenchymal origin:  heterogeneous appearance
  • May contain areas of osteoid, cartilage, calcification, fibrosis, necrosis, and hemorrhage


  • Long-term survival rates are 75-80%
  • Ppure fetal tumors have the best prognosis (outcome)
  • Small cell undifferentiated/anaplastic tumors have the worst prognosis

Diagnosic studies:

  • Labs: AFP (alpha fetoprotein) level
    • AFP abnormally elevated in 80% of newly diagnosed cases
    • Poorer prognosis if AFP is extremely high or low
    • Low AFP associated with small cell undifferentiated type, especially unresponsive to chemotherapy
    • Large, early fall after chemotherapy is a good prognostic indicator
  • Imaging
    • Ultrasound – preliminary imaging study
    • CT – quick and easy, usually without sedation, good for detecting pulmonary metastasis
    • MRI – More accurate at showing tumor margins, evaluating portal vein, and detecting residual tumor after surgical resection
    • MRA – demonstrates vascular anatomy and segmental involvement


  • Primary goal of treatment is surgical resection
  • 30-60% of patients present with resectable tumors clear margins
  • Of the initially unresectable tumors, 60-75% will decrease in size after chemotherapy and allow for surgical resection

Unresectable at presentation if:

  • Tumor crosses planned margins of resection
  • Resection may result in excessive bleeding
  • Invasion of hepatic blood vessels or the IVC
  • Disease is diffuse and multifocal


  • Vincristine
  • Adriamycin
  • Cyclophosphamide
  • 5-Fluorouracil

After Chemotherapy:
Monitor with CT and AFP after 2 to 4 cycles and resect if clear margins can be obtained.


  • Indicated for those patients with unresectable tumors after chemotherapy
  • Can be done with cadaveric liver donor or living liver donor
  • 1 year survival – 79%
  • 5 year survival – 69%
  • 10 year survival – 66%


  • Old Staging (CCG/POG) (post surgery staging)
    • Stage I – completely resected
    • Stage II – microscopic residual at margins of resected specimen
    • Stage III – partially resected, unresected specimen confined to liver, tumor spill during surgery, or + lymph nodes
    • Stage IV – distant metastatic disease
  • Current Staging – SIOP/PRETEXT (pre-surgical staging)
    • PRETEXT system
    • Divides liver into 4 sectors or quadrants
    • PRETEXT 1 involves 1 sector of the liver
    • PRETEXT 2 involves 2 sectors, etc.
    • PRETEXT 3  Tumor involves 3 sectors and 1 sector is free of tumor
    •       or tumor involves 2 sectors and 2 nonadjoining sectors are free of tumor
    • PRETEXT 4 Tumor involves all four sectors; there is no sector free of tumor.
    • V – involves the hepatic vein
    • P – involves the portal vein
    • C – involves the caudate lobe of liver
    • E – extrahepatic extension
    • M – distant metastasis

Emre S, McKenna GJ. “Liver Tumors in children”
Pediatr Transplantation 2004:8:632-38

Evans AE, Land VJ, Newton WA, Randolf JG, Sather HN, Tefft M. “Combination chemotherapy (vincristine, adriamycin, cyclophosphamide, and 5-fluorouracil) in the treatment of children with malignant hepatoma”

Mann JR, Kasthuri N, Raafat F, Pincott JR, Parkes SE, MuirKR, et al “Malignant hepatic tumours in children: incidence, clinical features and aetiology”
Paediatr Perinat Epidemiol 1990;4:276-89

Penn I. “Hepatic transplantation for primary and metastatic cancers of the liver”
Surgery 1991;110:726-34

Molmenti EP, Nagata D, Roden J, Squires R, Molmenti H, Casey D, et al. “Liver transplantation for hepatoblastoma in the pediatric population”
Transplant Proc 2001;33:1749

Raney B.
J Ped Hematol Oncol 1997;19:418-22

Tiao et al. “The current management of hepatoblastoma: a combination of chemotherapy, conventional resection, and liver transplantation”
J Pediatr 2005;146:204-11


Very graphic content related to the type of surgery, organs, procedures or trauma depicted in this tab.×