Update on Surveillance Guidelines in Emerging Wilms Tumor Predisposition Syndromes
Wilms tumors are commonly associated with predisposition syndromes. Many of these syndromes are associated with specific phenotypic features and are discussed in the related article from the AACR Pediatric Cancer Working Group. Guidelines for surveillance in this population were published in 2017, but since then several studies have identified new genes with recurrent pathogenic variants associated with increased risk for Wilms tumor development. In general, variants in these genes are less likely to be associated with other phenotypic features. Recently, members of the AACR Pediatric Cancer Working Group met to update surveillance guidelines for patients with a predisposition to Wilms tumors with a review of recently published evidence and risk estimates. Risk estimates for Wilms tumor for the more recently described genes are discussed here along with suggested surveillance guidelines for these populations. Several other emerging clinical scenarios associated with Wilms tumor predisposition are also discussed, including patients with family histories of Wilms tumor and no identified causative gene, patients with bilateral tumors, and patients with somatic mosaicism for chromosome 11p15.5 alterations. This perspective serves to update pediatric oncologists, geneticists, radiologists, counselors, and other health care professionals on emerging evidence and harmonize updated surveillance recommendations in the North American and Australian context for patients with emerging forms of Wilms tumor predisposition.
Authors
- Anita Villani (ORCID: https://orcid.org/0000-0003-3283-2197)
- Kristian W. Pajtler (ORCID: https://orcid.org/0000-0002-3562-6121)
- Garrett M. Brodeur (ORCID: https://orcid.org/0000-0002-6721-122X)
- Jack Brzezinski (ORCID: https://orcid.org/0000-0002-9475-0077)
- Lisa Diller (ORCID: https://orcid.org/0000-0002-4073-5509)
- Gaëlle Bougeard (ORCID: https://orcid.org/0000-0002-1475-0254)
- Jaclyn Schienda (ORCID: https://orcid.org/0000-0003-2543-8378)
- Kristin Zelley (ORCID: https://orcid.org/0000-0003-0970-1850)
- Jordan R. Hansford (ORCID: https://orcid.org/0000-0001-7733-383X)
- Rosanna Weksberg (ORCID: https://orcid.org/0000-0002-6501-4150)
- Kerri Becktell (ORCID: https://orcid.org/0000-0002-1935-5157)
- Surya P. Rednam (ORCID: https://orcid.org/0000-0001-8333-0758)
- Gail E. Tomlinson (ORCID: https://orcid.org/0000-0003-2579-1833)
- Wendy K. Kohlmann (ORCID: https://orcid.org/0000-0002-9134-9640)
- Jennifer M. Kalish (ORCID: https://orcid.org/0000-0003-1500-9713)
- Christian P. Kratz (ORCID: https://orcid.org/0000-0003-4120-5873)
- Andrea Schwarz Doria (ORCID: https://orcid.org/0000-0003-3202-0447)
- Lisa J. States (ORCID: https://orcid.org/0000-0003-0260-3975)
- Suzanne P. MacFarland (ORCID: https://orcid.org/0000-0001-5894-6403)
Institutions
- Children's Hospital of Philadelphia (US)
- Inserm (FR)
- The University of Texas at San Antonio Health Science Center (US)
- German Cancer Research Center (DE)
- Baylor College of Medicine (US)
- Medical College of Wisconsin (US)
- Heidelberg University (DE)
- Pennsylvania Hospital (US)
- Huntsman Cancer Institute (US)
- Women's and Children's Hospital (AU)
- Hospital for Sick Children (CA)
- University Hospital Heidelberg (DE)
- Medizinische Hochschule Hannover (DE)
- National Center for Tumor Diseases (DE)
- Hopp Children's Cancer Center Heidelberg (DE)
- South Australian Health and Medical Research Institute (AU)
- Durham VA Medical Center (US)
- Dana-Farber/Boston Children's Cancer and Blood Disorders Center (US)
- Université de Rouen Normandie (FR)
- University of Pennsylvania (US)
Publication Details
- Journal
- Clinical Cancer Research
- Published
- 2024-10-28
- DOI
- https://doi.org/10.1158/1078-0432.ccr-24-2488
- Citations
- 6
- Primary Topic
- Renal and related cancers
- Type
- article
- Field-Weighted Citation Impact
- 1.40
Funders
- Alexion Pharmaceuticals
- Servier