The pathophysiology of bilateral and multifocal Wilms tumors: What we can learn from the study of predisposition syndromes

N Welter, J Brzezinski, A Treece… - Pediatric Blood & …, 2023 - Wiley Online Library
Approximately 5% of patients with Wilms tumor present with synchronous bilateral disease.
The development of synchronous bilateral Wilms tumor (BWT) is highly suggestive of a …

Genetic and epigenetic features of bilateral Wilms tumor predisposition in patients from the Children's Oncology Group AREN18B5-Q

AJ Murphy, C Cheng, J Williams, TI Shaw… - Nature …, 2023 - nature.com
Developing synchronous bilateral Wilms tumor suggests an underlying (epi) genetic
predisposition. Here, we evaluate this predisposition in 68 patients using whole exome or …

TRIM28 congenital predisposition to Wilms' tumor: novel mutations and presentation in a sibling pair

C Moore, H Monforte, JK Teer… - Molecular …, 2020 - molecularcasestudies.cshlp.org
Wilms' tumor is the most common renal malignancy in children. In addition to staging,
molecular risk stratification, such as loss of heterozygosity (LOH) in Chromosomes 1 and 16 …

Recent advances in Wilms' tumor predisposition

JL Maciaszek, N Oak, KE Nichols - Human molecular genetics, 2020 - academic.oup.com
Abstract Wilms' tumor (WT), the most common childhood kidney cancer, develops in
association with an underlying germline predisposition in up to 15% of cases. Germline …

[PDF][PDF] Multiple pathways to Wilms tumor: how much is genetic?

K Pritchard-Jones, G Vujanic - Pediatric Blood and Cancer, 2006 - researchgate.net
Even before Knudson's two hit hypothesis was proven at a molecular level for
retinoblastoma, there was emerging evidence that the genetics of Wilms tumor involved …

Renal function in patients with Wilms tumor

RLP Romao, AJ Lorenzo - Urologic Oncology: Seminars and Original …, 2016 - Elsevier
Survival in patients with Wilms tumor (WT) is excellent compared with other pediatric
malignancies and adult renal tumors. Treatment-related long-term morbidity and mortality in …

[PDF][PDF] Wilms tumor overview

JS Dome, V Huff - Pagon, RA, 1993 - corpora.tika.apache.org
Disease characteristics. Wilms tumor (nephroblastoma) is an embryonal malignancy of the
kidney. Current models of Wilms tumor development propose that a genetic mutation …

Results of treatment for patients with multicentric or bilaterally predisposed unilateral Wilms tumor (AREN0534): a report from the Children's Oncology Group

PF Ehrlich, YY Chi, MM Chintagumpala, FA Hoffer… - Cancer, 2020 - Wiley Online Library
Background A primary objective of Children's Oncology Group study AREN0534 (Treatment
for Patients With Multicentric or Bilaterally Predisposed, Unilateral Wilms Tumor) was to …

A unique subset of low-risk Wilms tumors is characterized by loss of function of TRIM28 (KAP1), a gene critical in early renal development: A Children's Oncology …

AE Armstrong, S Gadd, V Huff, DS Gerhard, JS Dome… - PLoS …, 2018 - journals.plos.org
This study explores the genomic alterations that contribute to the formation of a unique
subset of low-risk, epithelial differentiated, favorable histology Wilms tumors (WT), tumors …

[HTML][HTML] Stratification of Wilms tumor by genetic and epigenetic analysis

RH Scott, A Murray, L Baskcomb, C Turnbull… - Oncotarget, 2012 - ncbi.nlm.nih.gov
Somatic defects at five loci, WT1, CTNNB1, WTX, TP53 and the imprinted 11p15 region, are
implicated in Wilms tumor, the commonest childhood kidney cancer. In this study we …