What are the symptoms of Wilms tumor (nephroblastoma)?

From the Guidelines

Wilms tumor symptoms typically include an abdominal mass or swelling that is often painless, which parents or doctors may notice during routine examinations, and early detection is crucial for successful treatment, so any unexplained abdominal swelling in a child should prompt immediate medical evaluation. Other common symptoms include abdominal pain, fever, blood in the urine (hematuria), high blood pressure, and general symptoms like fatigue, loss of appetite, or weight loss 1. Some children may develop constipation or have breathing difficulties if the tumor grows large enough to press on other organs. Occasionally, Wilms tumor can present with sudden abdominal pain and vomiting due to bleeding inside the tumor. It's essential to note that many children with Wilms tumor appear otherwise healthy, and the abdominal mass might be the only sign.

The symptoms develop because the tumor originates in the kidney and grows, potentially affecting kidney function and surrounding structures. According to recent guidelines, surveillance by renal ultrasound every 3 months is recommended for children with emerging Wilms tumor predisposition syndromes, such as those with TRIM28, REST, DIS3L2, and CTR9 gene variants, from diagnosis until at least 8 years of age 1. Wilms tumor most commonly affects children between ages 3-4 years, though it can occur at any age during childhood.

Key points to consider in the diagnosis and management of Wilms tumor include:

• Abdominal mass or swelling as a common presenting symptom
• Importance of early detection for successful treatment
• Surveillance recommendations for children with emerging Wilms tumor predisposition syndromes
• Genetic testing and counseling for children with bilateral Wilms tumors or those with a family history of the disease 1
• Ongoing tumor surveillance for children who have been treated for bilateral Wilms tumors, regardless of whether they have syndromic features or harbor a variant in a known predisposition gene 1.

From the Research

Wilms Tumor Symptoms

• The typical presenting features of Wilms tumor include:
  • Asymptomatic abdominal mass (most common) 2
  • Hematuria 2, 3
  • Pain 2
  • Fever 2
  • Hypertension 2, 3
• The nonspecific nature of symptoms can delay timely diagnosis and treatment 4
• Children with Wilms tumor often present acutely with hypertension which resolves in the short term after nephrectomy, but survivors have increased long-term risk of hypertension 3
• Abdominal mass or swelling without other signs or symptoms is a common clinical presentation 4

Diagnosis and Staging

• The diagnosis of Wilms tumor needs positive sonographic and computed tomography (CT) findings with histopathological confirmation 2
• CT or MRI examination of the abdomen can add additional information in the local staging of Wilms' tumours, including detection of nephroblastomatosis, contralateral tumours, tumour haemorrhage, abdominal lymph node enlargement, and inferior vena cava (IVC)/renal vein thrombus 5