NCCN Issues First-Ever Guidance for Pediatric Solid Tumor

Elizabeth Mullen, MD

The National Comprehensive Cancer Network (NCCN) has issued guidelines for the management of children with a rare type of kidney cancer referred to as Wilms Tumor, or nephroblastoma.¹

The document highlights evidence-based recommendations for how to maximize benefits while minimizing toxicities in this patient population. Notably, the guidance comprises critical information for providers who treat pediatric tumors, hematologic malignancies, and other blood disorders that may be encountered relatively infrequently. The guidelines also contain diagnostic information for primary care pediatricians to ensure that the cancer is identified early and managed in a way that results in fewer toxicities for the patients.

“Keeping up with all of the current literature for the broad array of children’s oncologic and hematologic malignancies can be daunting. The NCCN Guidelines for Wilms Tumor provide compiled, annotated, and vetted recommendations for diagnosis, treatment, and follow up—in keeping with NCCN’s goals for streamlining and improving care so that patients can live better lives,” Elizabeth Mullen, MD, of Dana-Farber/Boston Children’s Cancer and Blood Disorders Center, who is also a member of the NCCN Guidelines Panel for Wilms Tumor, stated in a press release.²

Wilms tumor is the most common cancer of the kidneys in children and it is known to affect those between the ages of 3 years and 4 years.³ In the majority of patients, the tumor will present in just 1 kidney, although it has been identified in both kidneys at the same time.

One factor that may increase the risk of Wilms tumor is African-American race; these children tend to be at a slightly higher risk for this cancer compared with other races. Additionally, Asian-American children may be at lower risk than children of other races. Another risk factor is having a familial history of the cancer.

Wilms tumor is also known to occur more commonly in children with aniridia or hemihypertrophy at birth; it has also been shown to occur as part of other rare syndromes like WAGR syndrome, Denys-Drash syndrome, and Beckwith-Wiedemann syndrome.

“The treatment of Wilms tumor is guided by the results of a series of sequential clinical trials that started back in the 1960s,” Frank M. Balis, MD, professor of pediatrics, Louis and Amelia Canuso Family Endowed Chair for Clinical Research in Oncology, of the Children’s Hospital of Philadelphia/Abramson Cancer Center at the University of Pennsylvania, and chair of the NCCN Guidelines Panel for Wilms Tumor, added in the release. “Treatment approaches have been refined through these clinical trials, making treatment selection more and more complex and nuanced.”

Balis added that treatment decisions should be based on clinical and biological risk factors, the presence of unilateral versus bilateral disease, and patient response to treatment.

References

1. NCCN clinical practice guidelines in oncology (NCCN guidelines): Wilms tumor. National Comprehensive Cancer Network. Accessed February 17, 2021.
2. New NCCN guidelines analyze evidence for cancer type found almost exclusively in children. News release. National Comprehensive Cancer Network. February 17, 2021. Accessed February 17, 2021. http://prn.to/2NfnfJ8
3. Wilms’ tumor. Mayo Clinic website. Updated April 24, 2020. Accessed February 17, 2021. http://mayocl.in/3qwE2G2