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Wilms tumor (nephroblastoma) – clinical and genetic aspects
Affiliations- Clinical Department of Paediatric Bone Marrow Transplantation, Oncology and Haematology, Faculty of Medicine, Wroclaw Medical University, Wroclaw, Poland
- Department of Genetics, Faculty of Medicine, Wroclaw Medical University, Wroclaw, Poland
- Clinical Department of Paediatric Nephrology, Faculty of Medicine, Wroclaw Medical University, Wroclaw, Poland
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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.
Keywords
Wilms tumor; genetics clinical; presentation; nephrological control
About this articleTitle
Wilms tumor (nephroblastoma) – clinical and genetic aspects
Journal
Nowotwory. Journal of Oncology
Issue
Article type
Review paper
Pages
259-264
Published online
2022-08-22
Page views
3990
Article views/downloads
345
DOI
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
References (29)- Bhutani N, Kajal P, Sharma U. Many faces of Wilms Tumor: Recent advances and future directions. Ann Med Surg (Lond). 2021; 64: 102202.
- Nelson MV, van den Heuvel-Eibrink MM, Graf N, et al. New approaches to risk stratification for Wilms tumor. Curr Opin Pediatr. 2021; 33(1): 40–48.
- Jemal A, Siegel R, Ward E, et al. Cancer statistics, 2006. CA Cancer J Clin. 2006; 56(2): 106–130.
- van den Heuvel-Eibrink MM, Hol JA, Pritchard-Jones K, et al. International Society of Paediatric Oncology — Renal Tumour Study Group (SIOP–RTSG). Position paper: Rationale for the treatment of Wilms tumour in the UMBRELLA SIOP-RTSG 2016 protocol. Nat Rev Urol. 2017; 14(12): 743–752.
- Han Q, Li K, Dong K, et al. Clinical features, treatment, and outcomes of bilateral Wilms' tumor: A systematic review and meta-analysis. J Pediatr Surg. 2018; 53(12): 2465–2469.
- Ruteshouser EC, Huff V. Familial Wilms tumor. Am J Med Genet C Semin Med Genet. 2004; 129C(1): 29–34.
- Dumoucel S, Gauthier-Villars M, Stoppa-Lyonnet D, et al. Malformations, genetic abnormalities, and Wilms tumor. Pediatr Blood Cancer. 2014; 61(1): 140–144.
- Seseke F, Gutjahr P, Kremens B. [Wilms tumor]. Urologe A. 2006; 45 Suppl 4: 235–238.
- Irtan S, Ehrlich PF, Pritchard-Jones K. Wilms tumor: "State-of-the-art" update, 2016. Semin Pediatr Surg. 2016; 25(5): 250–256.
- Siegel MJ, Chung EM. Wilms' tumor and other pediatric renal masses. Magn Reson Imaging Clin N Am. 2008; 16(3): 479–97, vi.
- Watson T, Oostveen M, Rogers H, et al. The role of imaging in the initial investigation of paediatric renal tumours. Lancet Child Adolesc Health. 2020; 4(3): 232–241.
- Janeczko M, Niedzielska E, Pietras W. Evaluation of Renal Function in Pediatric Patients After Treatment for Wilms' Tumor. Adv Clin Exp Med. 2015; 24(3): 497–504.
- Davidoff A. Wilmsʼ tumor. Curr Opin Pediatr. 2009; 21(3): 357–364.
- Termuhlen AM, Tersak JM, Liu Qi, et al. Twenty-five year follow-up of childhood Wilms tumor: a report from the Childhood Cancer Survivor Study. Pediatr Blood Cancer. 2011; 57(7): 1210–1216.
- Deng C, Dai R, Li X, et al. Genetic variation frequencies in Wilms' tumor: A meta-analysis and systematic review. Cancer Sci. 2016; 107(5): 690–699.
- Vujanić GM, Gessler M, Ooms AH, et al. International Society of Paediatric Oncology–Renal Tumour Study Group (SIOP–RTSG). The UMBRELLA SIOP-RTSG 2016 Wilms tumour pathology and molecular biology protocol. Nat Rev Urol. 2018; 15(11): 693–701.
- Pietras W. Advances and changes in the treatment of children with nephroblastoma. Adv Clin Exp Med. 2012; 21(6): 809–820.
- Chen H, Yang S, Qian C. Effectiveness of Nephron Sparing Surgery and Radical Nephrectomy in the Management of Unilateral Wilms Tumor: A Meta-Analysis. Front Oncol. 2020; 10: 1248.
- Groenendijk A, Spreafico F, de Krijger RR, et al. Prognostic Factors for Wilms Tumor Recurrence: A Review of the Literature. Cancers (Basel). 2021; 13(13).
- Breslow NE, Collins AJ, Ritchey ML, et al. End stage renal disease in patients with Wilms tumor: results from the National Wilms Tumor Study Group and the United States Renal Data System. J Urol. 2005; 174(5): 1972–1975.
- Interiano RB, Delos Santos N, Huang S, et al. Renal function in survivors of nonsyndromic Wilms tumor treated with unilateral radical nephrectomy. Cancer. 2015; 121(14): 2449–2456.
- Daw NC, Gregornik D, Rodman J, et al. Renal function after ifosfamide, carboplatin and etoposide (ICE) chemotherapy, nephrectomy and radiotherapy in children with Wilms tumour. Eur J Cancer. 2009; 45(1): 99–106.
- Diller L, Ghahremani M, Morgan J, et al. Constitutional WT1 mutations in Wilms' tumor patients. J Clin Oncol. 1998; 16(11): 3634–3640.
- Scott RH, Stiller CA, Walker L, et al. Syndromes and constitutional chromosomal abnormalities associated with Wilms tumour. J Med Genet. 2006; 43(9): 705–715.
- Wilde JCH, Godzinski J, Heij H, et al. Nephron sparing surgery (NSS) for unilateral wilms tumor (UWT): the SIOP 2001 experience. Pediatr Blood Cancer. 2014; 61(12): 2175–2179.
- Cost NG, Sawicz-Birkowska K, Kajbafzadeh AM, et al. A comparison of renal function outcomes after nephron-sparing surgery and radical nephrectomy for nonsyndromic unilateral Wilms tumor. Urology. 2014; 83(6): 1388–1393.
- Falcone MP, Pritchard-Jones K, Brok J, et al. Long-term kidney function in children with Wilms tumour and constitutional WT1 pathogenic variant. Pediatr Nephrol. 2022; 37(4): 821–832.
- Chu DI, Ehlayel AM, Ginsberg JP, et al. Kidney Outcomes and Hypertension in Survivors of Wilms Tumor: A Prospective Cohort Study. J Pediatr. 2021; 230: 215–220.e1.
- Green DM, Wang M, Krasin MJ, et al. Long-term renal function after treatment for unilateral, nonsyndromic Wilms tumor. A report from the St. Jude Lifetime Cohort Study. Pediatr Blood Cancer. 2020; 67(10): e28271.