Screening for Wilms tumor in high-risk individuals.

Recognition of the association between a variety of genetic syndromes and Wilms tumor has influenced physicians to develop tumor surveillance protocols based on empiric risk estimates. In recent years, clinical evaluation of high-risk individuals has been greatly refined and sensible screening protocols have been proposed. A continued effort to clinically characterize the Wilms tumor syndromes continues to be important, and a patient registry is currently being generated by one of the authors (CC). This registry will prospectively assess the occurrence of Wilms tumor in patients with high-risk phenotypes. All interested clinicians are encouraged to enroll their patients. As molecular genetic technologies refine our knowledge of factors leading to Wilms tumor, improved mechanisms of surveillance in high-risk individuals will be developed. Recent characterization of molecular mechanisms responsible for cases of BWS, isolated hemihypertrophy, DDS, familial Wilms tumor, and aniridia has already influenced the clinical management of high-risk patients and has become an important adjunct to clinical assessment in these patients. The involvement of clinical genetics in the coordination of clinical assessment, genetic testing, and tumor surveillance will provide better patient care and will promote the development of a multidisciplinary approach to Wilms tumor risk assessment. This collaboration between clinical genetics, molecular biology, oncology, and pediatrics should allow for a more precise understanding of tumor risks and will accelerate the understanding of factors involved in the genesis and progression of Wilms tumor.