1. Occurs in 2% of renal tumors.
2. Chromosomal deletion of 22q11-12 is present in these patients.
3. More than 50% of patients are less than 1 year of age at diagnosis.
4. Metastases found in lung, liver, and brain.
5. Poor prognosis; mortality is over 80%. Responds poorly to current chemotherapy and radiotherapy. No satisfactory treatment available but intensive chemotherapy (ICE therapy; see page 556) followed by sublethal chemotherapy and peripheral stem cell rescue has been utilized with some success in our clinic.
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