Wilms Tumor

What it is

Overview

Wilms tumor, or nephroblastoma, is the most common kidney cancer in children and one of the most successfully treated childhood cancers. It accounts for ~5% of all childhood cancers. Most children are diagnosed between ages 3 and 4. With modern multimodal treatment, the overall 5-year survival rate exceeds 90%.

Biology

How It Develops

Wilms tumor arises from embryonic kidney cells (metanephric blastema) that fail to differentiate normally. Mutations in WT1 (Wilms tumor suppressor gene), WTX, and CTNNB1 are found in a subset of cases. Bilateral disease occurs in about 5–8% of cases and is more often associated with genetic syndromes.

Warning signs

Symptoms

• —Abdominal mass or swelling (often painless)
• —Abdominal pain
• —Blood in the urine
• —High blood pressure
• —Fever
• —Loss of appetite

Detection

Diagnosis Methods

• —Abdominal ultrasound
• —CT of abdomen and chest
• —MRI
• —Biopsy (usually after surgical resection)
• —Genetic testing for WT1 and associated syndromes

Medical care

Treatment Options

• —Nephrectomy (kidney removal)
• —Chemotherapy (vincristine, actinomycin D, doxorubicin)
• —Radiation therapy for higher-stage disease
• —Bilateral disease: neoadjuvant chemo before bilateral partial nephrectomy

Data

Statistics

Prevention

Risk Factors

• —Beckwith-Wiedemann syndrome
• —WAGR syndrome (WT1 deletion)
• —Denys-Drash syndrome
• —Age 3–4 (peak incidence)

Further reading

Resources