NCCN publishes guidelines for standardized treatment of Wilms tumor

The National Comprehensive Cancer Network (NCCN) recently published its first-ever treatment guidelines for children in the United States who have nephroblastoma, a solid tumor cancer type known as “Wilms tumor.”

Wilms tumor is a solid cancerous tumor of the kidney that emerges from immature kidney cells. It accounts for 5% of all childhood cancers and typically occurs between ages one to five. About 500 new cases of Wilms tumor are found each year in the United States, affecting about one in every 10,000 children.

For most children with Wilms tumor, no clear cause is known. Certain environmental factors (such as contact with toxic chemicals) may increase the risk of developing this disease, but more research is needed. Wilms tends to be more common among Black children and is often caught in more advanced disease stages for this population. In rare cases, children with a number of genetic predisposition syndromes may get Wilms tumor; therefore, these children should be screened for Wilms tumor.

The new NCCN Guidelines contain evidence-based expert consensus recommendations for how to maximize benefits and minimize side effects for managing Wilms tumor. The guidelines contain crucial information, particularly for care providers who treat a full spectrum of pediatric tumors, hematologic malignancies, and other blood disorders (such as sickle cell), due to the relative infrequency of all of these conditions.

“Wilms tumor is rare, but highly treatable,” says Daniel M. Green, MD, an oncologist at St. Jude Children’s Research Hospital who served as vice-chair of the NCCN Guidelines Panel for Wilms tumor. “It’s important that we make sure these guidelines and knowledge of the signs and symptoms of Wilms tumor reach primary care physicians and pediatricians so they can help catch it early and save lives.”

The guidelines include important diagnostic information for primary care pediatricians to help ensure this cancer is caught early enough to benefit from high curability rates, and managed in a way that reduces side effects. Children often have abdominal swelling and/or a mass with or without other symptoms; however, they often appear healthy, which can delay diagnosis.

The guidelines also contain a warning for primary care physicians to be gentle if they feel a mass in the kidney area, especially if the children appear to be otherwise healthy. Wilms tumors can rupture if touched with too much force, causing the cancer to spread within the abdomen or elsewhere.

Andrew M. Davidoff, MD, chair of the Surgery Department and director of Surgical Research at St. Jude, served on the reviewing committee for the new guidelines.

“The significance of these guidelines is that treatment for Wilms tumor is now increasingly standardized and should be available to children throughout the United States, regardless of where they live,” Davidoff says.

See the full announcement from the NCCN.