Wilms' tumor is an ideal model to demonstrate how multi-modality treatment strategies have reduced disease mortality over the past three decades. More than 80percnt; of all patients are currently long-term survivors. Greater understanding of biology and awareness of clinical syndromes have led to more risk-based therapies. Although routine imaging provides adequate information for staging, advanced radiographic techniques, including spiral CT scanning and enhanced magnetic resonance imaging, can delineate improved anatomic detail. Recently, parenchymal sparing operations have been undertaken as long-term renal insufficiency after nephrectomy for Wilms' tumor has been more frequently recognized. Primary chemotherapy with delayed tumor resection is increasingly advocated for patients with bilateral disease, tumors with intravascular extension, or for those whose tumors are considered ldquo;inoperable.rdquo; Technical advances, including intraoperative ultrasonography, regional hypothermia, laser technology, and minimally invasive surgery, will influence future tumor resections. Nevertheless, primary nephrectomy with appropriate operative guidelines and systemic therapy remain important standards for management of sporadic unilateral Wilms' tumor.
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