scholarly journals Age distributions, birth weights, nephrogenic rests, and heterogeneity in the pathogenesis of Wilms tumor

2006 ◽  
Vol 47 (3) ◽  
pp. 260-267 ◽  
Author(s):  
Norman E. Breslow ◽  
J. Bruce Beckwith ◽  
Elizabeth J. Perlman ◽  
Anthony E. Reeve
Keyword(s):  
Wilms Tumor ◽  
Nephrogenic Rests

Adult Extrarenal Wilms Tumor Occurring in the Uterus

2001 ◽  
Vol 125 (8) ◽  
pp. 1081-1083 ◽  
Author(s):  
Ronald S. Muc ◽  
Wayne Grayson ◽  
Johannes J. Grobbelaar
Keyword(s):  
Differential Diagnosis ◽  
Genital Tract ◽  
Wilms Tumor ◽  
Female Genital Tract ◽  
Age Group ◽  
Important Differential Diagnosis ◽  
Nephrogenic Rests ◽  
Mullerian Tumor ◽  
Glomeruloid Structures ◽  
Female Genital

Abstract Five previous cases of extrarenal Wilms tumor (EWT) occurring in the uterus have been reported. The oldest patient was 22 years. We report a case of uterine EWT occurring in a 42-year-old woman. Histologically, there was typical triphasic differentiation, including epithelial, blastemal, and mesenchymal elements. The important differential diagnosis in this age group, the malignant mixed mullerian tumor, is excluded by the absence of glomeruloid structures and primitive tubules. The exact histogenesis of EWT is unknown but most likely relates to the presence of nephrogenic rests occurring in the female genital tract.

Nephrogenic rests located inside a retroperitoneal colonic duplication and their evolution into a Wilms tumor

2020 ◽  
Vol 67 (11) ◽  
Author(s):  
Cécile Picard ◽  
Pierre Leblond ◽  
Jean‐Pierre Pracros ◽  
Grégoire Schneider ◽  
Frédéric Hameury ◽  
...  
Keyword(s):  
Wilms Tumor ◽  
Colonic Duplication ◽  
Nephrogenic Rests

scholarly journals Nephrogenic rests and nephroblastomatosis: literature review and clinical reports

2020 ◽  
Vol 7 (3) ◽  
pp. 119-124
Author(s):  
L. I. Shats ◽  
B. V. Kondratiev ◽  
M. B. Belogurova
Keyword(s):  
Literature Review ◽  
Wilms Tumor ◽  
Renal Parenchyma ◽  
The Other ◽  
Wilms Tumour ◽  
Different Types ◽  
Nephrogenic Rests ◽  
Metanephric Blastema

Nephrogenic rests and nephroblastomatosis describe persistence of embryonic renal parenchyma (metanephric blastema) beyond 36 weeks of gestation, when nephrogenesis is normally complete. This persistent metanephric blastema may transform into the Wilms tumor, and are detected in 30–40 % of unilateral nephroblstoma and nearly 100 % in bilateral cases. The risk of Wilms tumour is increased in any type of nephrogenic rests/nephroblastomatosis but it is higher in infants and in patients with intralobar nephrogenic rests. We cite below several studies described different types of nephrogenic rests, their connection with nephroblastoma development and the details of diagnostic. We refer to clinical examples, in that regard. Two cases are presented: bilateral diffuse hyperplastic perilobar nephroblastomatosis in one child and an infant with combination of perilobar nephrogenic rests in one kidney and Wilms tumour in the other kidney.

Nephrogenic rests mimicking Wilms' tumor on CT

2004 ◽  
Vol 34 (2) ◽  
pp. 152-155 ◽  
Author(s):  
Pedram Argani ◽  
John P. Gearhart ◽  
Stanley S. Siegelman ◽  
Naveen Subhas
Keyword(s):  
Wilms Tumor ◽  
Nephrogenic Rests

Metanephric Adenoma, Nephrogenic Rests, and Wilms' Tumor

2001 ◽  
Vol 25 (10) ◽  
pp. 1290-1296 ◽  
Author(s):  
Trudie E. Muir ◽  
John C. Cheville ◽  
Donna J. Lager
Keyword(s):  
Wilms Tumor ◽  
Metanephric Adenoma ◽  
Nephrogenic Rests

Bilaterally multicentric synchronous Wilms' tumor: Successful conservative treatment despite persistence of nephrogenic rests

1997 ◽  
Vol 28 (6) ◽  
pp. 420-423 ◽  
Author(s):  
Jacinto J. Regalado ◽  
Maria M. Rodriguez ◽  
Stuart Toledano
Keyword(s):  
Conservative Treatment ◽  
Wilms Tumor ◽  
Nephrogenic Rests

Bilateral Nephroblastic Tumors and a Complex Renal Vascular Anomaly in a Patient With a Mosaic RASopathy: Novel Histopathologic Features and Molecular Insights

2021 ◽  
pp. 109352662098650
Author(s):  
Jonathan C Slack ◽  
Marie-Anne Bründler ◽  
Caitlin A Chang ◽  
Renee Perrier ◽  
Lucie Lafay-Cousin ◽  
...  
Keyword(s):  
Molecular Mechanisms ◽  
Wilms Tumor ◽  
Vascular Anomaly ◽  
Driver Mutations ◽  
Bone Lesions ◽  
Activating Mutations ◽  
Nephrogenic Rest ◽  
Sclerotic Bone Lesions ◽  
Renal Vascular ◽  
Nephrogenic Rests

Mosaic RASopathies are an emerging group of disorders characterized by mosaic or post-zygotic activating mutations in genes of the RAS/MAPKinase signaling pathway. The phenotype is highly variable, ranging from limited or localized forms to cases with a syndromic presentation with extensive or multiorgan involvement, and also overlaps with other mosaic disorders. While there are several reports of malignancies in patients with mosaic RASopathies, specifically rhabdomyosarcoma and transitional urothelial carcinoma, the lifetime risk and molecular mechanisms that lead to the development of malignancies remain unclear. We report a 22-month-old boy with a somatic RASopathy due to an underlying KRAS p.G12D mutation who presented with a large unilateral epidermal nevus, asymmetric lower limb overgrowth with lytic and sclerotic bone lesions, capillary malformation, bilateral nephrogenic rests and Wilms tumors, and a novel complex renal vascular anomaly that resembles Fibro-Adipose Vascular Anomaly (FAVA). This report further expands the phenotypic spectrum of somatic RASopathies, and discusses the potential phenotypic and pathogenetic overlap with PIK3CA-related overgrowth disorders, specifically CLOVES. The occurrence of a secondary cancer hotspot mutation ( FBXW7 p.R479G ) in the Wilms tumor, but not the associated nephrogenic rest, moreover suggests that additional driver mutations are involved in the development of Wilms tumor in somatic overgrowth disorders.

Perilobar Nephrogenic Rests and Chromosome 22

2011 ◽  
Vol 14 (6) ◽  
pp. 485-492 ◽  
Author(s):  
Reena MdZIN ◽  
Marianne Phillips ◽  
Caitlin Edwards ◽  
Ashleigh Murch ◽  
Adrian Charles
Keyword(s):  
In Situ Hybridization ◽  
Fluorescent In Situ Hybridization ◽  
Wilms Tumor ◽  
Microsatellite Analysis ◽  
Chromosome 22 ◽  
Hybridization Probe ◽  
Genetic Changes ◽  
Preferential Loss ◽  
Nephrogenic Rests

Perilobar nephrogenic rests (NR) are precursor lesions that may display genetic changes similar to their associated Wilms tumor (WT). Two patients presented with WT, both with perilobar NR and 1 with bilateral, multifocal metachronous WT. Both patients' WT displayed monosomy 22 as the predominant cytogenetic change, and the constitutional cytogenetic analysis was normal. The purpose of our study was to identify at what stage in the morphologic progression from NR to WT the monosomy 22 occurred by using a fluorescent in situ hybridization probe for chromosome 22 in the subtypes of perilobar NR and WT present in both cases. Section and core fluorescent in situ hybridization with a chromosome 22 probe was performed on formalin-fixed, paraffin-embedded tissues containing WT and perilobar NR. We also performed fluorescent-based microsatellite analysis on some of the WT in the bilateral case to determine whether there was a preferential loss of the same allele of chromosome 22. Sclerotic and dormant perilobar NR showed a rate of monosomy 22 of only approximately 30%, but this increased to approximately 50% in hyperplastic and adenomatous NR. Monosomy 22 was present in 60%-80% of nuclei in WT. Microsatellite analysis showed loss of homozygosity, with preferential loss of the same allele of chromosome 22 in the tumors examined. There are differences in the rate of detection of monosomy 22 in perilobar NR and WT, suggesting loss of chromosome 22 in the progression of perilobar NR to WT in a subset of tumors.

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