Hepatoblastoma

Hepatoblastoma is the most common primary malignant liver tumour in children under four years of age who usually present with painless abdominal mass and raised AFP. It is tumour of embryonic origin.

Most cases are seen during the first 18 months of life and diagnosis in adulthood is exceedingly rare. Occasionally the tumour may be diagnosed antenatally 2. There is a recognised slight male preponderance with a M:F ratio of up to ≈3:2. There may also be predilection towards the right lobe of the liver.

Most children present with abdominal distension or an asymptomatic  palpable abdominal mass. However, other presenting symptoms include 1:

  • abdominal pain
  • anorexia
  • vomiting
  • jaundice
  • pyrexia
  • anaemia
  • back pain
  • pseudo precocious puberty (due to chronic GnRH secretion)

Macroscopically hepatoblastomas are usually relatively well circumscribed large masses, usually single, with heterogeneous cut surface 10.

Histological classification of hepatoblastoma is complicated with multiple subtypes identified. The notable subtypes include:

  • epithelial: most common
    • fetal: best prognosis
    • embryonal
    • small cell undifferentiated: worst prognosis
  • mixed type: epithelial and mesenchymal. Calcifications are more common in mixed type

It is important to note that with the possible exception of small cell undifferentiated subtype, prognosis is independent of histology when adjusted for stage gender and age 10.

For full classification please refer to hepatoblastoma histological classification.

Associations

Most hepatoblastomas are seen sporadically, however, it has been known to be associated with:

Markers

Serum alpha feto protein (AFP) levels are frequently elevated (90% of cases 6).

Staging

See: hepatoblastoma staging. Caval involvement often indicates unresectable disease.

Plain radiograph

Abdominal x-rays are nonspecific, typically demonstrating a right upper quadrant mass. Calcifications are visible in 10% of cases 1,4.

Ultrasound

On ultrasound, hepatoblastomas appear as predominantly echogenic soft tissue mass. In larger tumours heterogeneity and variable echogenicity is common. Even when large, they tend to be relatively well defined 7. Intralesional calcifications may be visible as areas of shadowing 4,7.

CT

Usually seen as a well defined heterogeneous mass, which is usually hypoattenuating compared to surrounding liver 11.  Frequently there are with areas of necrosis and haemorrhage. Chunky, dense calcifications may be seen in approximately 40% of cases 11.

CT is also able to evaluate the lungs for metastases and for nodal enlargement.

MRI

MRI is superior to CT in defining tumour margins, vessel involvement and adenopathy 1.

  • T1: generally hypointense
  • T1 C+ (Gd): can show heterogeneous enhancement
  • T2
    • generally hyperintense compared to liver
    • areas of necrosis and haemorrhage are common
Angiography
  • tend to be hypervascular

Surgical resection is the treatment of choice although preoperative chemotherapy may be used to reduce tumour bulk. Chemotherapy is also employed in following surgical resections. If the tumour is resectable then liver transplantation provides a cure, as long as no metastatic disease is present. The lungs are a relatively frequent site of metastases.

Overall there is 65-70% long term survival 10, however, prognosis depends on staging:

  • stage I: can expect very good long term survival, of up to 100% with combined chemotherapy and surgery 8,12
  • stage II: 75-80%
  • stage III: 65%
  • stage IV: 0-27% 10

General imaging differential considerations include:

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Article information

rID: 8376
Section: Pathology
Tag: liver
Synonyms or Alternate Spellings:
  • Hepatoblastomas

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Cases and figures

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    Case 1
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    Case 2: T1 C+
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    Case 3: with calcification
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    Case 3
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    Case 4
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    Case 5
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    Case 6: epithelial type
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    Case 7
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    Case 8: with pulmonary metastasis
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    Case 9: Hepatoblastoma with lung metastases
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