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Originates from the embryonal kidney
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Pathologically contains renal tissue with various degrees of
differentiation
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Affects about 1 in 10,000 live births
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60%
present before the age of three years
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10%
tumours are bilateral
·
The
presentation is with an:
o
Abdominal mass (90%)
o
Abdominal pain (20%)
o
Haematuria (30%)
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Diagnosis can be confirmed by CT scan
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40%
have metastatic spread at presentation but do not prevent cure
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Treatment is with nephrectomy and postoperative chemotherapy and
radiotherapy
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Stage 1 disease (localised to kidney) has 3-year survival of >80%
·
Stage 4 disease (haematogenous spread) has 3-year survival less than
30%
Neuroblastoma
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Arises from neural crest tissue - usually adrenal medulla or
sympathetic ganglia
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Show a range of malignancy from benign ganglioneuroma to malignant
neuroblastoma
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Tumours in children are usually malignant
·
75%
are abdominal
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25%
arise in thorax, pelvis or neck
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Affects about 1 in 8,000 live births
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Usually occur in first five years of life
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Clinical presentation depends on site of tumour and presence of
metastases
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Bone and pulmonary metastases are relatively common
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Symptoms often due to metastases include:
o
Pallor, weight loss, irritability (40%)
o
Limb pain and hypertension (15%)
o
Abdominal mass or pain (30%)
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90%
have increased urinary VMA and HVA
·
Pain abdominal x-ray often shows diffused speckled calcification
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Diagnosis can be confirmed by CT scan
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Treatment is with surgery and post-operative radiotherapy
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Prognosis is best in children presenting before 2 years
·
Stage 1 disease (localised to kidney) has 3-year survival of >90%
·
Stage 4 disease (haematogenous spread) has 3-year survival less than
30%
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