Rationale for the treatment of children with CCSK in the UMBRELLA SIOP–RTSG 2016 protocol

Gooskens, Saskia L.; Graf, Norbert; Furtwängler, Rhoikos; Spreafico, Filippo; Bergeron, Christophe; Ramírez-Villar, Gema L.; Godziński, Jan; Rübe, Christian; Janssens, Geert O.; Vujanić, Gordan; Leuschner, Ivo; Coulomb-L’Herminé, Aurore; Smets, Anne; Camargo, Beatriz de; Stoneham, Sara; Pritchard‐Jones, Kathy; Heuvel‐Eibrink, Marry M. van den

doi:10.1038/nrurol.2018.14

Cited by 46 publications

(67 citation statements)

References 80 publications

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“…This is a reasonable analysis as although there are no trials comparing the different regimens for CCSK, there are no survival differences between the UK (where the CCLG advocates AVD preoperatively if a CSSK is identified) and Germany, where biopsy is rarely employed and thus an AV regimen is de facto used or between SIOP and NWTS/COG approaches. 26 Ultimately, the percentage of cases in which biopsy will change management is lower than the incidence of procedural complications previously reported. 12 Our data suggest that biopsy in children over 6 months is not effective in avoiding unnecessary chemotherapy in children with benign tumors but it is clearly effective in identifying RCCs (an important diagnosis to make to avoid unnecessary chemotherapy and allow timely surgical resection).…”

Section: Discussionmentioning

confidence: 94%

The diagnostic accuracy and clinical utility of pediatric renal tumor biopsy: Report of the UK experience in the SIOP UK WT 2001 trial

Jackson

Williams

Brok

et al. 2019

Pediatric Blood & Cancer

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Introduction The International Society of Paediatric Oncology (SIOP) protocols recommend pre-operative chemotherapy appropriate for Wilms tumours (WT) in children with renal tumours aged ≥6 months, reserving biopsy for ‘atypical’ cases. The Children’s Cancer and Leukaemia Group (CCLG) joined the SIOP-WT-2001 study but continued the national practice of biopsy at presentation. Method Retrospective study of concordance between locally reported renal tumour biopsies and central pathology review nephrectomy diagnoses of children enrolled by CCLG centres in SIOP-WT-2001 Study. Results Biopsy reports were available for 552/787 children with unilateral tumours. 36/552 (6.5%) were non–diagnostic: 2 normal tissue, 12 necrotic, 9 insufficient sample and 13 indeterminate results (disproportionately non-WTs). The sensitivity and specificity of biopsy to identify tumours that did not require SIOP empirical pre-operative chemotherapy were 86.0% and 99.6%, respectively. 13/548 (2.4%) biopsy results were discordant with nephrectomy; non-WTs other than renal cell carcinoma and clear cell sarcoma (CCSK) were poorly recognised. In children aged 6-119 months, 480/518 (91.6%) had WT or nephroblastomatosis. 5/518 (1%) had benign tumours, only one diagnosed on biopsy. Biopsy results correctly changed clinical management in 25/518 (4.8%), including identifying 19/20 CCSKs, but would have led to overtreatment in 5/518 (1%) or undertreatment in 4/518 (0.8%). In children aged ≥10 years, biopsy correctly changed management in 5/19 (26%) cases with no discordance. Conclusion Biopsy is less effective at identifying non-WTs than WTs and rarely changes management in younger children. Biopsy should be reserved in SIOP protocols for children ≥10 years and in younger children with clinical or radiological features inconsistent with WT.

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Section: Discussionmentioning

confidence: 94%

The diagnostic accuracy and clinical utility of pediatric renal tumor biopsy: Report of the UK experience in the SIOP UK WT 2001 trial

Jackson

Williams

Brok

et al. 2019

Pediatric Blood & Cancer

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“…9,10 Current international treatment guidelines include the European UMBRELLA International Society of Pediatric Oncology (SIOP)-RTSG 2016 protocol and the North American NWTS-Children's Oncology Group (COG) guidlines. 24 The SIOP protocol advocates for preoperative chemotherapy, whereas the NWTS-COG guidelines specify primary surgery. Both of these treatment strategies have shown similar survival data.…”

Section: Treatment and Prognosismentioning

confidence: 99%

“…Both of these treatment strategies have shown similar survival data. 24 Treatment recommendations depend on the tumor stage. In the current SIOP protocol, preoperative chemotherapy with actinomycin and vincristine is indicated for stages I to III disease, with the addition of doxorubicin for stage IV disease.…”

Section: Treatment and Prognosismentioning

confidence: 99%

Clear Cell Sarcoma of the Kidney

Aldera

Pillay

2019

Archives of Pathology &Amp; Laboratory Medicine

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Clear cell sarcoma of the kidney is an uncommon malignant pediatric renal neoplasm that typically presents in the 2- to 3-year age group and has a propensity for aggressive behavior and late relapses. Histologically, this tumor exhibits a great diversity of morphologic patterns that can mimic most other pediatric renal neoplasms, often leading to confusion and misdiagnosis. Until recently, adjunct immunohistochemical and molecular genetic tests to support the diagnosis were lacking. The presence of internal tandem duplications in BCL-6 coreceptor ( BCOR) and a translocation t(10;17) creating the fusion gene YWHAE-NUTM2B/E have now been well accepted. Immunohistochemistry for BCOR has also been shown to be a sensitive and specific marker for clear cell sarcoma of the kidney in the context of pediatric renal tumors. Improved intensive chemotherapy regimens have influenced the clinical course of the disease, with late relapses now being less frequent and the brain having overtaken bone as the most common site of relapse.

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“…Overall survival of children with Wilms tumour has greatly improved. Yet, few effective treatment options exist for high-risk Wilms and most non-Wilms tumours [5][6][7] . Moreover, survivors have significant risks of late effects of the harsh treatment regimen 8 .…”

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confidence: 99%

An organoid biobank for childhood kidney cancers that captures disease and tissue heterogeneity

et al. 2020

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Kidney tumours are among the most common solid tumours in children, comprising distinct subtypes differing in many aspects, including cell-of-origin, genetics, and pathology. Preclinical cell models capturing the disease heterogeneity are currently lacking. Here, we describe the first paediatric cancer organoid biobank. It contains tumour and matching normal kidney organoids from over 50 children with different subtypes of kidney cancer, including Wilms tumours, malignant rhabdoid tumours, renal cell carcinomas, and congenital mesoblastic nephromas. Paediatric kidney tumour organoids retain key properties of native tumours, useful for revealing patient-specific drug sensitivities. Using single cell RNAsequencing and high resolution 3D imaging, we further demonstrate that organoid cultures derived from Wilms tumours consist of multiple different cell types, including epithelial, stromal and blastemal-like cells. Our organoid biobank captures the heterogeneity of paediatric kidney tumours, providing a representative collection of well-characterised models for basic cancer research, drug-screening and personalised medicine.

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Rationale for the treatment of children with CCSK in the UMBRELLA SIOP–RTSG 2016 protocol

Cited by 46 publications

References 80 publications

The diagnostic accuracy and clinical utility of pediatric renal tumor biopsy: Report of the UK experience in the SIOP UK WT 2001 trial

The diagnostic accuracy and clinical utility of pediatric renal tumor biopsy: Report of the UK experience in the SIOP UK WT 2001 trial

Clear Cell Sarcoma of the Kidney

An organoid biobank for childhood kidney cancers that captures disease and tissue heterogeneity

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