Renal tumours of childhood

Charles, Adrian; Gm, Vujanić; Berry, P J

doi:10.1046/j.1365-2559.1998.00344.x

Cited by 61 publications

(49 citation statements)

References 121 publications

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“…In WT57, a cystic nephroma-like area showed 7p LOH, as did the bulk of the tumour. This supports the contention that cystic nephroma is closely related to Wilms' tumour, and that cystic nephroma-like areas within a WT should be regarded as a part of the tumour, or possibly a precursor lesion (Charles et al, 1998b). In contrast, in WT40, where there were bilateral tumours, the second contralateral tumour did not show 7p LOH, whereas both tumours had LOH at 11p15.…”

Section: supporting

confidence: 75%

Loss of heterozygosity at 7p in Wilms’ tumour development

et al. 2000

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Chromosome 7p alterations have been implicated in the development of Wilms' tumour (WT) by previous studies of tumour cytogenetics, and by our analysis of a constitutional translocation (t(1;7)(q42;p15)) in a child with WT and radial aplasia. We therefore used polymorphic microsatellite markers on 7p for a loss of heterozygosity (LOH) study, and found LOH in seven out of 77 informative WTs (9%). The common region of LOH was 7p15–7p22, which contains the region disrupted by the t(1;7) breakpoint. Four WTs with 7p LOH had other genetic changes; a germline WT1 mutation with 11p LOH, LOH at 11p, LOH at 16q, and loss of imprinting of IGF2 . Analysis of three tumour-associated lesions from 7p LOH cases revealed a cystic nephroma-like area also having 7p LOH. However, a nephrogenic rest and a contralateral WT from the two other cases showed no 7p LOH. No particular clinical phenotype was associated with the WTs which showed 7p LOH. The frequency and pattern of 7p LOH demonstrated in our studies indicate the presence of a tumour suppressor gene at 7p involved in the development of Wilms' tumour. © 2000 Cancer Research Campaign

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Section: supporting

confidence: 75%

Loss of heterozygosity at 7p in Wilms’ tumour development

et al. 2000

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“…Greater than 80 % of Wilms' tumors are found in children younger than 5 years old, with an average age of 3.5 years [ 1 ]. Rarely, 0.16 % of Wilms' tumors are seen in neonates, and on very few occasions it occurs in adults [ 7 ].…”

Section: Defi Nitionmentioning

confidence: 99%

“…In the USA, there is a lower incidence in Hispanic/Latino compared with non-Hispanic children (RR = 0.78, 95 % confi dence interval = [0.64, 0.95]) [ 9 ]. The incidences in Chinese and American black children are 2.5 per million and 10.9 per million, respectively [ 7 ]. In Britain, the incidence is lower among Asian children than Caucasian children (RR = 0.51, p < 0.05) but higher among West Indian children (RR = 2.55, p < 0.05) [ 9 ].…”

Section: Defi Nitionmentioning

confidence: 99%

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Malignant Renal Tumors

Shehata

Naguib

Lin

et al. 2014

Pediatric Malignancies: Pathology and Imaging

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Renal tumors account for approximately 6-7 % of all pediatric cancers, with Wilms' tumor being the most common malignancy [ 1 , 2 ]. Current classifi cation and staging criteria for renal tumors, aided by advances in molecular and cytogenetic abnormalities, have allowed accurate diagnostic, staging, and therapeutic protocols [ 2 ], elucidated new categories of renal neoplasms, and created more inclusive classifi cation (Table 10.1 ) [ 2 , 3 ]. Overview of Imaging FeaturesThe imaging evaluation of a suspected pediatric renal mass should always begin with ultrasound. Sonography is readily available, can be performed without sedation and intravenous contrast, and does not expose the child to ionizing radiation. If a renal mass is identifi ed on ultrasound, further evaluation of the renal vessels and inferior vena cava (IVC) with both gray scale and color Doppler images are required to detect vascular extension, present in up to 10 % of cases [ 4 ]. Further evaluation with contrast enhanced computed tomography (CT) or magnetic resonance imaging (MRI) is also required for local staging of the mass prior to any intervention, as per the guidelines of the Children's Oncology Group (COG). Either modality can be used, depending on institutional availability and expertise [ 5 ].In keeping with the As Low As Reasonably Achievable (ALARA) principle regarding ionizing radiation exposure, pre-contrast and multiphase contrast enhanced CT images are not required for diagnosis or staging of pediatric renal tumors [ 6 ]. A single-phase study in the portal venous phase (approximately 50 s after contrast injection) suffi ciently stages a renal tumor and delineates relevant anatomy. Multiplanar CT reconstructions or multiplanar scanning with MRI can confi rm the renal origin of the mass and assess its relationship to vital structures such as the renal vessels. If a partial nephrectomy is being considered, multiphase images are helpful to determine the relationship of the mass to the renal vessels and collecting system. A CT of the chest is required in all malignant renal tumors to evaluate for potential lung metastasis. Wilm's Tumor Defi nitionWilms' tumor ( syn: nephroblastoma), a malignant neoplasm originating from nephrogenic blastemal cells, is the second most common malignant, solid extracranial tumor in children [ 1 , 7 , 8 ]. It is a traditional blastematous tumor, exhibiting various stages of embryonic development and multiple lines of differentiation. Clinical Features and EpidemiologyIn children ranging from 0 to 15 years of age, Wilms' tumor occurs in seven to ten cases per million annually (1 in 10,000 children) and accounts for approximately 95 % of renal tumors, and comprises 6-7 % of all pediatric tumors [ 7 , 9 , 10 ]. It is the fi fth most common pediatric tumor and the second most common intraabdominal tumor in children [ 11 ].

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“…Wilms tumor (nephroblastoma; WT) is a pediatric renal tumor that annually affects 1:10,000 children between the ages 0 and 15 (Charles et al, 1998). When treated according to the International Society of Pediatric Oncology (SIOP) protocol (Vujanic et al, 2002), patients are pretreated with chemotherapy to shrink the tumor before it is surgically removed and a histopathologic diagnosis is made.…”

Section: Introductionmentioning

confidence: 99%

High‐resolution molecular cytogenetic analysis of Wilms tumors highlights diagnostic difficulties among small round cell kidney tumors

Stewénius

Jin

Øra

et al. 2008

Genes Chromosomes & Cancer

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Many solid tumors exhibit characteristic gene fusions, which are reflected by balanced translocations at the cytogenetic level. These changes might be useful diagnostic and prognostic tools. In Wilms tumor (WT, nephroblastoma) no fusions genes or recurrent balanced translocations have been described thus far. To screen for cryptic balanced translocations, we have analyzed 17 renal neoplasms, histopathologically classified as WT, by a combination of G-banding, multicolor FISH, and subtelomeric FISH. This approach revealed several submicroscopic chromosomal aberrations and three different seemingly balanced translocations, resulting in a heterozygous deletion of HACE1, an EWSR1/ERG fusion, and an EWSR1/FLI1 fusion, respectively. As EWSR1 rearrangements are known to be a characteristic of Ewing tumors (ET), our findings illustrate the diagnostic problems regarding small cell kidney tumors and strongly argue for the need of adjuvant diagnostic techniques in this group of neoplasms. In summary, our genomic screening approach proved efficient in finding structural chromosomal aberrations. The fact that no recurrent translocations were found in the WTs of this study argues against the presence of a frequent pathognomonic translocation in this disease entity.

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Renal tumours of childhood

Cited by 61 publications

References 121 publications

Loss of heterozygosity at 7p in Wilms’ tumour development

Loss of heterozygosity at 7p in Wilms’ tumour development

Malignant Renal Tumors

High‐resolution molecular cytogenetic analysis of Wilms tumors highlights diagnostic difficulties among small round cell kidney tumors

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