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Fig. 8.48 Hepatoblastoma in a patient 3 years of age. The T2 weighted MRI shows a liver mass that histologically corresponds to fetal epithelial hepatoblastoma.

Table 8.02

Age distribution of hepatic tumours in young patients. Data are from the Armed Forces Institute of Pathology (AFIP), Washington, DC (U.S.A.)

Fig. 8.48 Hepatoblastoma in a patient 3 years of age. The T2 weighted MRI shows a liver mass that histologically corresponds to fetal epithelial hepatoblastoma.

tion {1233}. Magnetic resonance imaging (MRI) along with CT can help differentiate hepatoblastoma from infantile haeman-gioendothelioma, mesenchymal hamar-toma, and hepatocellular carcinoma by demonstrating cystic or vascular features peculiar to each lesion {1999}. MRI may also be used to characterize epithelial and mesenchymal components of hepa-toblastoma {1533}.

Macroscopy

Hepatoblastomas vary in size from 5 to 22 cm in diameter and from 150 to 1,400 g in weight. Single and multiple lesions may be well circumscribed, the edge of the lesion being separated from the normal liver by an irregular pseudocapsule. Pure fetal hepatoblastomas have the tan-brown colour of normal liver, while mixed hepatoblastomas display a variety of colours from brown to green to white. The lesions are often nodular and bulge from the cut surface. Areas of necrosis and haemorrhage are usually present and may appear as soft or gelatinous, brown to red tissue {1837}.

Tumour spread

At clinical manifestation, 40-60% of hepatoblastomas are either very large or involve both lobes to the extent that they are considered unresectable {1839}. Preoperative chemotherapy, however, reduces the size of the lesion in nearly 85% of these patients to a size that renders it resectable. Tumour spread includes local extension into the hepatic

Fig. 8.49 Epithelial hepatoblastoma presenting as a large, well demarcated lesion with central haemorrhage.

veins and inferior vena cava. The lung is the most frequent site of metastases; approximately 10-20% of patients have pulmonary metastases when first diagnosed. Hepatoblastomas also spread to bone, brain, ovaries, and the eye {179, 1600, 619, 463}.

Histopathology

Hepatoblastomas display a distinct variety of histological patterns that may be present in varying proportions. Some tumours are composed entirely of uniform fetal epithelial cells or small undifferentiated cells, while others contain a variety of tissue types including hepatic fetal epithelial and embryonal cells, fibrous connective tissue, osteoid-like material, skeletal muscle fibers, nests of squamous epithelial cells, and cells with melanin pigment.

Pure fetal epithelial differentiation

Accounting for nearly one third of cases, the fetal epithelial pattern is composed of thin trabeculae of small cuboidal cells resembling the hepatocytes of the devel-

Table 8.03

Staging of Hepatoblastoma according to the Children's Cancer Study Group (CCSG) classification.

Table 8.03

Staging of Hepatoblastoma according to the Children's Cancer Study Group (CCSG) classification.

Stage I

Complete resection

Stage II

Microscopic residual

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