Wilms' Tumor

(Nephroblastoma; Kidney Tumor)

Definition

Wilms' tumor is a type of kidney cancer that mainly affects children. In most cases, Wilms' tumor affects only 1 of the 2 kidneys.
Kidneys
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Causes

In the fetus, some of the cells that are supposed to grow into mature kidneys stay as fetal kidney cells. Clusters of these cells sometimes remain after birth. If they do not mature within 3 or 4 years, they may grow without control or order. A large mass of these cells may develop into a Wilms' tumor.

Risk Factors

A small percentage of children with Wilms' tumors have inherited an abnormal gene from one parent. This gene increases the chance that kidney cells will turn into a Wilms' tumor. Having a family member with a Wilms' tumor also increases the risk in a child. Also, certain birth defects increase the risk of a Wilms' tumor. These include:
  • WAGR—a syndrome that includes:
    • W ilms' tumor
    • A niridia—completely or partly missing the colored part of the eyes called the iris
    • G enitourinary abnormalities—defects of the kidneys, urinary tract, penis, scrotum, testicles, clitoris, or ovaries
    • Mental R etardation
  • Beckwith-Wiedemann syndrome—larger than normal tongue and internal organs; 1 arm or leg may be larger than the other
  • Perlman syndrome—large fetal size, renal dysplasia, Wilms' tumor, islet cell hypertrophy, multiple congenital anomalies, and mental retardation
  • Denys-Drash syndrome—absence of the penis, scrotum, and testicles
  • Hemihypertrophy—1 side of the body larger is than the other
  • Other genetic defects, including defects on chromosome 11.
Many children with Wilms' tumors do not have any known inherited gene changes or birth defects. Researchers do not know why these children have some kidney cells that do not mature properly.

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