Characteristics and survival of 750 children diagnosed with a renal tumor in the first seven months of life: A collaborative study by the SIOP/GPOH/SFOP, NWTSG, and UKCCSG Wilms tumor study groups

Heuvel‐Eibrink, Marry M. van den; Grundy, Paul E.; Graf, Norbert; Pritchard‐Jones, Kathy; Bergeron, Christophe; Patte, Catherine; Tinteren, Harm van; Rey, Annie; Langford, Carolyn F.; Anderson, J. R.; Kraker, Jan de

doi:10.1002/pbc.21389

Cited by 155 publications

(104 citation statements)

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“…The reason for upfront nephrectomy is that, compared with older children, a higher proportion of renal tumours in infants are congenital mesoblastic nephroma or malignant rhabdoid tumours that either need surgery alone (congenital mesoblastic nephroma) or alternative chemotherapy at the outset (more intensive chemotherapy than actinomycin D and vincristine) 44,45 . Percutaneous cutting needle biopsy is recommended in instances of stage IV disease or when immediate surgery is deemed difficult.…”

Section: Infant Wilms Tumoursmentioning

confidence: 99%

“…Percutaneous cutting needle biopsy is recommended in instances of stage IV disease or when immediate surgery is deemed difficult. Postoperative chemotherapy for Wilms tumour is similar in infants to that in older children who underwent direct nephrectomy, with adjustment of drug doses according to age and body weight based on the experience from previous SIOP studies 44 .…”

Section: Infant Wilms Tumoursmentioning

confidence: 99%

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Rationale for the treatment of Wilms tumour in the UMBRELLA SIOP–RTSG 2016 protocol

et al. 2017

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Section: Infant Wilms Tumoursmentioning

confidence: 99%

Section: Infant Wilms Tumoursmentioning

confidence: 99%

Rationale for the treatment of Wilms tumour in the UMBRELLA SIOP–RTSG 2016 protocol

et al. 2017

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“…This is only partly explained by the fact that the occurrence of anaplasia increases with age (12); even in patients with favorable histology, older age seems to be associated with less favorable outcome. It still needs to be determined what the exact age threshold is at which outcome starts to deteriorate.…”

Section: Generalmentioning

confidence: 99%

“…Anaplasia is only very rarely seen in WT diagnosed during the first year of life and is also rare in the second year of life (12). Nevertheless, even in the group of patients with favorable histology, older age seems to be correlated with a higher risk of relapse and death, although prognostic factors such as stage or histology seem to be more powerful (7).…”

Section: Age In Correlation With Other Prognostic Factorsmentioning

confidence: 99%

The Clinical Relevance of Age at Presentation in Nephroblastoma

et al. 2016

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Licence: This open access article is licensed under Creative Commons Attribution 4.0 International (CC BY 4.0). http://creativecommons.org/licenses/by/4.0/ Users are allowed to share (copy and redistribute the material in any medium or format) and adapt (remix, transform, and build upon the material for any purpose, even commercially), as long as the authors and the publisher are explicitly identified and properly acknowledged as the original source. AbstractThe most important prognostic factors for Wilms tumor (WT) patients seem to be stage, histological subtype, and 1p/16q loss of heterozygosity (LOH) in chemotherapy-naive WTs. 24Over the last decade, age at diagnosis also was suggested to be an important risk factor for WT recurrence in Children's Oncology Group (COG), United Kingdom (UK), and International Society of Pediatric Oncology (SIOP) studies. Several studies have analyzed age as a prognostic factor; these studies revealed age <2 years as a favorable prognostic factor, while age >4 years has been described as an adverse prognostic factor. In adults (>18 years of age), WT represents less than 1% of all diagnosed renal tumors; therefore, diagnosis of WT in adults is often unexpected and poorly recognized, thereby inducing treatment delay with subsequent adverse outcome. One explanation for the higher risk of recurrence with increasing patient age is the higher frequency of anaplasia at higher age. Other suggested reasons are delay in diagnosis, advanced tumor stage at presentation, and intrinsically different biological behaviors. Whether age is really an independent risk factor, and whether age is a stronger prognostic factor than stage, histology, and LOH 1p/16q, needs to be further explored. This may provide some insight into whether older patients need to be treated more intensively, as is already advised for adult WT patients.

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“…Over time significant overlap between approaches has evolved for defined risk groups [*4]. SIOP and COG/NWTS recommendations for renal tumors are concordant for the following clinical scenarios: immediate nephrectomy is advocated for those < 7 mo, due to the high likelihood of non-WT histology [5]; preoperative chemotherapy is utilized for bilateral WT (Stage V). Both cooperative group approaches agree that involvement of abdominal lymph nodes constitute stage III disease [*6].…”

Section: Diagnosis and Treatmentmentioning

confidence: 99%

Pediatric genitourinary tumors

Castellino

McLean

2007

Current Opinion in Oncology

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Purpose of review-We will review the 2007/2008 literature on pediatric genitourinary tumors.Recent findings-Newly identified constitutional epigenetic defects in Wilms tumor (WT) genes extends the understanding of WT risks in children lacking syndromic features, and adds to the complexity of the pathogenesis of these tumor suppressor genes. Pediatric renal cell carcinoma (RCC) has distinct molecular characteristics and clinical associations from the adult counterpart. The pathway from PAX3-FKHR translocation to the development of rhabdomyosarcoma (RMS) tumors has been further elucidated.Summary-Therapeutic strategies continue to be driven by developments in molecular diagnostics in pediatric GU tumors.

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Characteristics and survival of 750 children diagnosed with a renal tumor in the first seven months of life: A collaborative study by the SIOP/GPOH/SFOP, NWTSG, and UKCCSG Wilms tumor study groups

Cited by 155 publications

References 21 publications

Rationale for the treatment of Wilms tumour in the UMBRELLA SIOP–RTSG 2016 protocol

Rationale for the treatment of Wilms tumour in the UMBRELLA SIOP–RTSG 2016 protocol

The Clinical Relevance of Age at Presentation in Nephroblastoma

Pediatric genitourinary tumors

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