Bilateral Wilms tumour: a review of clinical and molecular features

Charlton, Jocelyn; Irtan, Sabine; Bergeron, Christophe; Pritchard‐Jones, Kathy

doi:10.1017/erm.2017.8

Cited by 87 publications

(94 citation statements)

References 81 publications

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“…Synchronous bilateral Wilms tumours (stage V) occur in ~5-8% of patients, and these children are more likely to have an underlying genetic predisposition 26 . These patients are treated with preoperative chemotherapy for 6-12 weeks and nephron-sparing surgery (NSS) is considered by the surgical panel, taking into account tumour response to chemotherapy, to spare as much renal function as possible.…”

Section: Histological Classification Of Bilateral Wilms Tumourmentioning

confidence: 99%

The UMBRELLA SIOP–RTSG 2016 Wilms tumour pathology and molecular biology protocol

et al. 2018

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Paediatric renal tumours account for ~6% of all paediatric malignant tumours, of which ~90% are Wilms tumours (nephroblastoma) 1. Other renal non-Wilms tumours are rare entities and include mesoblastic nephroma, clear cell sarcoma of the kidney, rhabdoid tumour of the kidney, renal cell carcinoma and few other, even rarer tumour types 1. The Renal Tumour Study Group of the International Society of Paediatric Oncology (SIOP-RTSG) has developed a new study for all renal tumours of childhood, the UMBRELLA SIOP-RTSG 2016 protocol (the UMBRELLA protocol) 2,3 , on the basis of the experiences from SIOP-2001 and the UK Improving Population Outcomes for Renal Tumours of Childhood (IMPORT) 2013 studies 2-4. The aim of the UMBRELLA protocol is to collect all clinical, biological and outcomes data from children with primary renal tumours in a comprehensive data registry, with central review of diagnostics (radiology, patho logy and surgery), standardized biobanking and precise treatment recommendations for the most common paediatric renal tumours. Molecular biology research within the protocol is primarily focused on the validation of the prognostic value of 1q gain, which might lead to a more personalized treatment approach (Box 1). Consequently, short-term and long-term outcomes might be improved for all children with renal tumours by increasing survival, but also by reducing treatment in specific subgroups, resulting in diminished direct and late adverse effects. Timely genetic analysis and step-wise extension to additional targets such as TP53 (refs 5-7) or several of the newly identified driver candidates for stratification and inclusion of liquid biopsies might help to reach this goal 8-10. The UMBRELLA protocol includes updated guidelines for pathologists for the handling and processing of tissue as well as criteria that are important for postoperative histological classification, staging and treatment stratification. These recommendations were established by a consensus of pathology experts within the SIOP-RTSG (chaired by G. M. Vujanić and I. Leuschner).

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Section: Histological Classification Of Bilateral Wilms Tumourmentioning

confidence: 99%

The UMBRELLA SIOP–RTSG 2016 Wilms tumour pathology and molecular biology protocol

et al. 2018

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“…9 The proportion of bilateral WT (8.9%) we found was slightly higher than in previous reports (5-8%). 7,8 This might indicate improved diagnostic imaging procedure, improved reporting, or improved identification of bilateral tumours.…”

Section: Wilms Tumourmentioning

confidence: 99%

Incidence of childhood renal tumours: An international population‐based study

Nakata

Colombet

Stiller

et al. 2020

Intl Journal of Cancer

103

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Malignant renal tumours represent 5% of childhood cancers and include types with likely different aetiology: Wilms tumour (WT), rhabdoid renal tumour, kidney sarcomas and renal carcinomas. WT is the most common renal tumour in children, previously shown to vary internationally and with ethnicity. Using the comprehensive database of the International Incidence of Childhood Cancer study (IICC), we analysed global variations and time trends in incidence of renal tumour types in children (age 0-14 years) and adolescents (age 15-19 years). The results were presented by 14 world regions, and five ethnic groups in the US. We included 15 320 renal tumours in children and 800 in adolescents reported to the 163 contributing registries during 2001-2010. In children, agestandardised incidence rate (ASR) of renal tumours was 8.3 per million (95% confidence interval, CI = 8.1, 8.4); it was the highest in North America and Europe (9-10 per million) and the lowest in most Asian regions (4-5 per million). In the US, Blacks had the highest ASR (10.9 per million, 95% CI = 10.2, 11.6) and Asian and Pacific Islanders the lowest (4.4 per million, 95% CI = 3.6, 5.1). In adolescents, age-specific incidence rate of renal tumours was 1.4 per million (95% CI = 1.3, 1.5). WT accounted for over 90% of all renal tumours in each age from 1 to 7 years and the proportion of renal carcinomas increased gradually with age.

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“…The review of the diagnostic imaging identified a total of 205 nodules. The median number of nodules per patient was four 2–10 . The radiological classification based on the criteria described above identified WT in 110 nodules (54%), NR in 78 nodules (38%), and there were 17 doubtful nodules (8.3%).…”

Section: Resultsmentioning

confidence: 99%

“…Actively proliferating perilobar and intralobar nephrogenic rests (PLNR, ILNR) and its diffuse presentation defined as nephroblastomatosis (NBM) can challenge the diagnosis because their radiological patterns can appear very similar to those of WT 4,5 . Distinguishing WT from their precursors is particularly important in children with bilateral disease, when nephron‐sparing surgery (NSS) is discussed and preservation of functioning renal tissue becomes mandatory 6–8 . Therefore, the purpose of this study is to compare the preoperative imaging features of kidney nodules with their final histology in order to check the concordance between radiology and histology.…”

Section: Introductionmentioning

confidence: 99%

Wilms tumors and their precursors: Radiological diagnosis versus histology

Dzhuma

Pointe

Coulomb

et al. 2020

Pediatric Blood & Cancer

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The radiological distinction of Wilms tumor (WT) nodules from nephrogenic rests (NR) in patients with multifocal unilateral WT or bilateral disease is challenging. The study aims to compare the radiology assessment of kidney nodules with their final histology in 48 patients. The final histology of 118 nodules corresponded to the initial radiological diagnosis while 40 (25%) nodules were misdiagnosed, 20 being initially diagnosed WT on imaging were proved to be NR at histology. The size of nodules at diagnosis might help to distinguish WT from NR before surgery. Homogeneity did not seem to be a key feature.

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Bilateral Wilms tumour: a review of clinical and molecular features

Cited by 87 publications

References 81 publications

The UMBRELLA SIOP–RTSG 2016 Wilms tumour pathology and molecular biology protocol

The UMBRELLA SIOP–RTSG 2016 Wilms tumour pathology and molecular biology protocol

Incidence of childhood renal tumours: An international population‐based study

Wilms tumors and their precursors: Radiological diagnosis versus histology

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