Learn About

Wilms’ Tumor

About Wilms’ Tumor

Learn about Wilms’ Tumor


Wilms’ tumor, also known as nephroblastoma, is a rare kind of kidney cancer that usually happens in young kids, but it can occasionally affect older children or teens too. It starts when some cells in the kidney grow out of control and form a lump or mass. Most of the time, Wilms’ Tumor only affects one kidney, and it’s usually found when someone notices a swelling or lump in the belly. Other symptoms might include stomach pain, blood in the urine, feeling tired, or a fever. Other possible symptoms include abdominal pain, blood in the urine (hematuria), fever, nausea, or high blood pressure.

The exact cause of Wilms’ tumor is not fully understood, but genetic mutations and abnormalities in certain genes, such as WT1 and WT2, play a significant role in its development. It can occur sporadically or as part of genetic syndromes like WAGR syndrome, Denys-Drash syndrome, Pearlman or Beckwith-Wiedemann syndrome. Diagnosis usually involves imaging studies like ultrasound or CT scans and is confirmed with a biopsy or surgical removal of the tumor. Treatment typically includes a combination of surgery, chemotherapy, and sometimes radiation therapy, depending on the stage and spread of the tumor. With early detection and appropriate treatment, the prognosis for Wilms’ tumor is generally very good, with a high survival rate, especially for young children.

Wilms’ tumor is the most common kidney cancer in children

It primarily affects children under the age of 5 and accounts for about 90% of pediatric kidney cancer cases. However, it is very rare in teens, and young adults.

It is often associated with genetic syndromes, or can be sporadic 

Conditions such as WAGR syndrome (Wilms’ tumor, Aniridia, Genitourinary anomalies, and intellectual disability), Denys-Drash syndrome, Pearlman, and Beckwith-Wiedemann syndrome increase the risk of developing Wilms’ tumor. Many sporadic Wilms’ tumors show mutations in genes that regulate kidney development, nephrogenic rests, or environmental or unknown prenatal factors. In short, most non-syndromic Wilms’ tumors appear to arise from random errors in gene regulation during kidney formation in utero.

Approximately 1.3% of all Wilms’ cases are in teens

Wilms’ tumor is extremely rare in adolescents. According to data from the U.S. Cancer Statistics database covering 2003–2020, only 106 cases of Wilms’ tumor were diagnosed in individuals aged 15–19 years, representing about 1.3% of all cases in the United States during that period. 

For adolescents aged 12-18 years in the United States, Wilms’ tumor remains an exceptionally rare condition. While specific data for this age group is limited, we can infer similar approximate figures based on available statistics.

Fewer than 10 new cases are diagnosed annually in the USA among individuals aged 15–19.