Nephroblastoma

Definition:

Nephroblastoma, also called Wilms tumor (WT), is the most common renal tumor of childhood. It can present as a single nodule, as multifocal unilateral lesions or as bilateral tumours. Typically, nephroblastoma comprises three histological components namely blastemal, epithelial and stromal. WT1, a zinc-finger transcription factor, was identified as the first nephroblastoma gene. Several other genes including CTNNB1, WTX, and TP53 have also been implicated in various stages of tumorigenesis of nephroblastoma.

References:

[1]. B E Weissman, et al. Introduction of a normal human chromosome 11 into a Wilms' tumor cell line controls its tumorigenic expression. Science. 1987 Apr 10;236(4798):175-80. [Content Brief]

[2]. Brigitte Royer-Pokora, et al. Genetics of pediatric renal tumors. Pediatr Nephrol. 2013 Jan;28(1):13-23. [Content Brief]

[3]. Changkai Deng, et al. Genetic variation frequencies in Wilms' tumor: A meta-analysis and systematic review. Cancer Sci. 2016 May;107(5):690-9. [Content Brief]

[4]. Francesca Di Renzo, et al. The murine Pou6f2 gene is temporally and spatially regulated during kidney embryogenesis and its human homolog is overexpressed in a subset of Wilms tumors. J Pediatr Hematol Oncol. 2006 Dec;28(12):791-7. [Content Brief]

[5]. G R M White, et al. Somatic glypican 3 (GPC3) mutations in Wilms' tumour. Br J Cancer. 2002 Jun 17;86(12):1920-2. [Content Brief]

[6]. Marry M. van den Heuvel-Eibrink, et al. Wilms Tumor [Internet]. [Content Brief]

[7]. S Reid, et al. Biallelic BRCA2 mutations are associated with multiple malignancies in childhood including familial Wilms tumour. J Med Genet. 2005 Feb;42(2):147-51. [Content Brief]

[8]. Shazia S Mahamdallie, et al. Mutations in the transcriptional repressor REST predispose to Wilms tumor. Nat Genet. 2015 Dec;47(12):1471-4. [Content Brief]

[9]. Turki Al-Hussain, et al. Wilms tumor: an update. Adv Anat Pathol. 2014 May;21(3):166-73. [Content Brief]

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