Type:
Educational Exhibit
Keywords:
Neoplasia, Metastases, Surgery, Chemotherapy, MR, CT, Paediatric, Oncology, Liver
Authors:
G. Sapouridis1, M. Sidiropoulou1, A. Stefanidou1, T. Nimas2, E. Papakonstantinou2, D. Koliouskas1, A. Anastasiou1; 1Thessaloniki/GR, 2Thessaloniki /GR
DOI:
10.1594/ecr2018/C-1785
Background
Hepatoblastoma is the most common primary liver malignancy in children (infancy to about 5 years old),
arising from primitive embryonic liver cells.
Hepatoblastoma affects white children more frequently than black children,
and is more common in boys than girls up to about age 5,
when the gender difference disappears.
Although the exact cause of liver cancer is unknown,
there are a number of genetic conditions that are associated with an increased risk for developing hepatoblastoma.
They include:
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Prematurity and low fetal birth weight.
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Beckwith-Wiedemann syndrome: This syndrome is characterized by a combination of Wilms tumor,
kidney failure,
genitourinary malformations and gonad (ovaries or testes) abnormalities.
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Familial adenomatous polyposis and Gardner syndrome: This is a group of rare inherited diseases of the gastrointestinal tract.
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Hemihypertrophy: This condition is the faster growth of one limb on one side of the body in comparison with the other side.
The signs and symptoms of hepatoblastoma often depend on the size of the tumor and whether it has spread to other parts of the body.
Symptoms may include:
Most common site for spread is lung and less brain and bone.
Histological classification of hepatoblastoma is complicated with multiple subtypes including epithelial (fetal,
embryonal and small cell undifferentiated) and mixed type (epithelial and mesenchymal).
There are a number of different staging systems of HB: PRETEXT grouping system of paediatric liver tumors and Intergroup staging system (specific for HB).
PRETreatment EXTent of tumor (PRETEXT) system Fig. 1 (pre-surgical staging) divides liver into 4 sectors or quadrants :
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PRETEXT 1 involves 1 sector of the liver.
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PRETEXT 2 involves 2 sectors.
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PRETEXT 3 tumor involves 3 sectors and 1 sector is free of tumor or tumor involves 2 sectors and 2 non-adjoining sectors are free of tumor.
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PRETEXT 4 Tumor involves all four sectors; there is no sector free of tumor.
Intergroup staging system (post-surgery staging) includes:
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Stage I – completely resected
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Stage II – microscopic residual at margins of resected specimen
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Stage III – partially resected,
unresected specimen confined to liver,
tumor spill during surgery,
or + lymph nodes
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Stage IV – distant metastatic disease