An embryonal neoplasm of the kidney which usually presents in children between the ages of 1 and 5 years as a mass of the upper abdomen or flank. It may be associated with such anomalies as aniridia, Beckwith-Wiedemann syndrome, urinary tract defects, hemihypertrophy, and chromosomal deletion. Its workup includes CT of the abdomen and chest and ultrasound which are important to rule out involvement of the renal vein or IVC. Surgical treatment is the primary mode of therapy and involves clamping the renal vein first to prevent venous embolization of tumor cells. Lymph nodes are sampled at the time of resection to assist with staging, which will determine if chemotherapy or radiation therapy will be needed. Patients with disease limited to the kidney which is completely excised, and treated with chemotherapy.
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