open access

Vol 72, No 4 (2022)
Review paper
Published online: 2022-08-22
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Wilms tumor (nephroblastoma) – clinical and genetic aspects

Małgorzata Janeczko-Czarnecka1, Ryszard Ślęzak2, Wojciech Pietras1, Katarzyna Kilis Pstrusinska3
DOI: 10.5603/NJO.2022.0040
·
Nowotwory. Journal of Oncology 2022;72(4):259-264.
Affiliations
  1. Clinical Department of Paediatric Bone Marrow Transplantation, Oncology and Haematology, Faculty of Medicine, Wroclaw Medical University, Wroclaw, Poland
  2. Department of Genetics, Faculty of Medicine, Wroclaw Medical University, Wroclaw, Poland
  3. Clinical Department of Paediatric Nephrology, Faculty of Medicine, Wroclaw Medical University, Wroclaw, Poland

open access

Vol 72, No 4 (2022)
Genetics and oncology
Published online: 2022-08-22

Abstract

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.

Abstract

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.

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Keywords

Wilms tumor; genetics clinical; presentation; nephrological control

About this article
Title

Wilms tumor (nephroblastoma) – clinical and genetic aspects

Journal

Nowotwory. Journal of Oncology

Issue

Vol 72, No 4 (2022)

Article type

Review paper

Pages

259-264

Published online

2022-08-22

Page views

87

Article views/downloads

52

DOI

10.5603/NJO.2022.0040

Bibliographic record

Nowotwory. Journal of Oncology 2022;72(4):259-264.

Keywords

Wilms tumor
genetics clinical
presentation
nephrological control

Authors

Małgorzata Janeczko-Czarnecka
Ryszard Ślęzak
Wojciech Pietras
Katarzyna Kilis Pstrusinska

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