Wilms tumor (nephroblastoma) – clinical and genetic aspects

Nephroblastoma (Wilms tumor – WT) is the most common kidney tumor among the pediatric population, fifth among malignant neoplasms and third among solid tumors. The most common type of WT is sporadic and unilateral. WT occurs either as an isolated, nonsyndromic WT or as syndromic one belonging to the spectrum of a variety of genetic syndromes. Molecular genetic testing should be considered in nonsyndromic WT and include a multigene panel or whole exome sequencing (WES); in syndromic cases single-gene testing, DNA methylation panel and chromosomal microarray. Outcomes of treatment in WT patients remain very good, but there are still subgroups with poor prognosis and increased relapse rates, especially in the blastemic and disseminated anaplasia types. WT survivors have increased risk of chronic kidney disease (CKD). They need further follow-up, not only by oncologists but also by nephrologists, to preserve kidney function or slow down CKD progression.