Nephroblastoma (Wilms Tumor) – Understanding a common Childhood Kindey Cancer

What is Nephroblastoma?

Wilms tumor, also known as nephroblastoma, is the most common type of kidney cancer in children13. It typically affects children under the age of 5, with a peak incidence between 2 and 3 years13. In high-income countries, the 5-year survival rate for Wilms tumor exceeds 90%, but in low- and middle-income countries like Kenya, the survival rate is less than 50%13.

Key Points

• Wilms tumor is a highly treatable childhood cancer with a good prognosis when diagnosed and treated early13.
• Treatment protocols in Kenya consist of preoperative chemotherapy, surgery, and postoperative chemotherapy, with the duration of treatment depending on the stage of the disease13.
• Survival rates for Wilms tumor in Kenya have improved over the years, but challenges remain in ensuring that all patients receive comprehensive treatment13.

Prevalence/Incidence

• Wilms tumor accounts for about 95% of all childhood kidney cancers3.
• In Kenya, a retrospective study conducted at a large teaching and referral hospital found that 92 patients were diagnosed with Wilms tumor between 2013 and 20161.

Risk Factors

• The exact cause of Wilms tumor is unknown, but certain genetic conditions, such as WAGR syndrome and Denys-Drash syndrome, are associated with an increased risk3.

Signs and Symptoms

• The most common symptom of Wilms tumor is a painless abdominal mass, which may be accompanied by abdominal pain, fever, and high blood pressure3.

Prevention Strategies

• There are no known prevention strategies for Wilms tumor, but early detection and prompt treatment are crucial for improving outcomes13.

Screening Modalities

• Diagnostic tests for Wilms tumor may include imaging studies such as ultrasound, CT scan, or MRI, as well as a biopsy to confirm the diagnosis3.

Diagnosis

• In Kenya, 69% of patients diagnosed with Wilms tumor presented with high-stage disease, which can negatively impact treatment outcomes1.

Treatment and Management

• Treatment for Wilms tumor in Kenya typically involves a combination of preoperative chemotherapy, surgery, and postoperative chemotherapy13.
• Patients who received comprehensive therapy, including radiation, had a 2-year event-free survival rate of 86%, which is approaching the survival rates seen in high-income countries1.

Despite the challenges faced in treating Wilms tumor in Kenya, such as late presentation and treatment abandonment, outcomes have improved over the years13. Ensuring that all patients receive comprehensive treatment and working on earlier diagnosis strategies can further enhance survival rates for children with Wilms tumor in Kenya1.