Fifty years of clinical and research studies for childhood renal tumors within the International Society of Pediatric Oncology (SIOP)

Graf, Norbert; Bergeron, Christophe; Brok, Jesper; Chowdhury, Tanzina; Furtwängler, Rhoikos; Gessler, Manfred; Godziński, Jan; Pritchard‐Jones, Kathy; Ramírez-Villar, Gema L.; Rübe, Christian; Sandstedt, Bengt; Schenk, Jens‐Peter; Spreafico, Filippo; Sudour-Bonnange, Hélène; Tinteren, Harm van; Verschuur, Arnauld; Vujanić, Gordan; Heuvel‐Eibrink, Marry M. van den

doi:10.1016/j.annonc.2021.08.1749

Cited by 16 publications

(16 citation statements)

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“…Since 1971, seven studies and trials conducted by the SIOP-RTSG (formerly known as SIOP Nephroblastoma Group) have contributed to improvements in the diagnostics and management of WT. 15 In the most recent completed trial (SIOPWT2001) reported here, the number of participating centers expanded to 251 from 26 countries, including Brazil, a large upper-middle–income country. 13 The SIOPWT2001 study protocol has been adapted for use in several low- and middle-income countries to improve survival chances in these settings.…”

Section: Discussionmentioning

confidence: 99%

International Comparisons of Clinical Demographics and Outcomes in the International Society of Pediatric Oncology Wilms Tumor 2001 Trial and Study

Neto

Camargo

Ramírez-Villar

et al. 2022

JCO Global Oncology

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PURPOSE International comparisons of patient demographics, tumor characteristics, and survival can shed light on areas for health care system improvement. The International Society of Pediatric Oncology Wilms Tumor 2001 trial/study registered patients through national clinical study groups in Western Europe and Brazil. This retrospective post hoc analysis of the International Society of Pediatric Oncology Wilms Tumor 2001 database aims to make visible and suggest reasons for any variations in outcomes. METHODS All patients with unilateral Wilms tumor (WT), age > 6 months, treated with preoperative chemotherapy as per protocol, and registered between 2001 and 2011 were eligible. Countries were grouped to give comparable case numbers and geographical representation. Cox univariable and multivariable (MVA) statistics were applied, with the German collaborative group (Gesellschaft für Pädiatrische Onkologie und Hämatologie—Austria, Germany, and Switzerland) as reference for hazard ratios for event-free survival (EFS) and overall survival (OS). RESULTS A total of 3,176 eligible patients were registered from 24 countries assigned into six groups. Age and histologic risk group distribution were similar across all groupings. The distribution of WT stage varied by country grouping, with 14.9% (range, 11.1%-18.2%) metastatic at diagnosis. Median follow-up was 78.9 months. For localized WT, 5-year EFS varied from 80% (Brazilian group) to 91% (French group; P < .0001), retaining significance only for Brazil in MVA ( P = .001). Five-year OS varied from 89% (Brazilian group) to 98% (French group; P < .0001). In MVA, only superior OS in France was significant ( P = .001). Five-year EFS/OS for stage IV did not vary significantly. High-risk histology and tumor volume at surgery were significantly associated with increased risk of death in MVA for metastatic disease. CONCLUSION International benchmarking of survival rates from WT within a large trial/study database has demonstrated statistically significant differences. Clinical interpretation should take account of variation in tumor stage but also treatment factors.

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Section: Discussionmentioning

confidence: 99%

International Comparisons of Clinical Demographics and Outcomes in the International Society of Pediatric Oncology Wilms Tumor 2001 Trial and Study

Neto

Camargo

Ramírez-Villar

et al. 2022

JCO Global Oncology

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“…Given the standard to start treatment with preoperative chemotherapy without histological confirmation, referral to an experienced pediatric radiology center for review at this stage is recommended to confirm the local radiology report and to identify cases that might benefit from a different treatment approach 25 . Nonetheless, for renal lesions as well as imaging of potential pulmonary metastases, both the COG and SIOP‐RTSG aim for centralized radiology review in children that will be enrolled on therapeutic protocols 26–28 …”

Section: Imaging Of Renal Tumors By the Cog And Siop‐rtsgmentioning

confidence: 99%

Similarities and controversies in imaging of pediatric renal tumors: A SIOP‐RTSG and COG collaboration

Beek

Artunduaga

Schenk

et al. 2022

Pediatric Blood & Cancer

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Malignant renal tumors are rare in children, and Wilms tumors (WTs) are the most common subtype. Imaging plays an essential role in the diagnosis, staging, and followup of these patients. Initial workup for staging is mainly performed by cross-sectional imaging modalities such as computed tomography (CT) and magnetic resonance imaging (MRI). Imaging approach within the two core international groups, the Children's Oncology Group (COG, North America) and the International Society of Pediatric Oncology -Renal Tumor Study Group (SIOP-RTSG, Europe), differs. Whereas abdominal ultrasound (US) is used for the initial diagnosis of a suspected pediatric renal tumor globally, COG protocols support the use of CT or MRI for locoregional staging, contrary to the preference for MRI over CT for abdominopelvic evaluation within the SIOP-RTSG. The purpose of this manuscript is to summarize current imaging approaches, highlighting differences and similarities within these core international groups, while focusing on future innovative efforts and collaboration within the HARMONICA initiative.

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“…Biallelic expression of imprinted genes or loss of imprinting (LOI) is frequently observed in human cancers ( Jelinic and Shaw, 2007 ), and is an early event for some tumors, such as the childhood Wilms’ tumor (WT) ( Graf et al, 2021 ). The imprinting control regions (ICR) are differentially methylated (DMR) in a parent-of-origin manner, resulting in a monoallelic expression of the clustered genes.…”

Section: Introductionmentioning

confidence: 99%

“…The imprinting control regions (ICR) are differentially methylated (DMR) in a parent-of-origin manner, resulting in a monoallelic expression of the clustered genes. LOI at the H19/IGF2 locus, for example, is associated with WT in overgrowth syndromes ( Graf et al, 2021 ), and other cancers such as bladder ( Byun et al, 2007 ) and colorectal ( Hidaka et al, 2018 ). LOI of KvDMR1, INPP5Fv2-DMR and RB1-DMR is also implicated in the pathogenesis of cancer ( Rumbajan et al, 2013 ).…”

Section: Introductionmentioning

confidence: 99%

Epigenetic reprogramming in cancer: From diagnosis to treatment

Costa

Sales

Pinheiro

et al. 2023

Front. Cell Dev. Biol.

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Disruption of the epigenetic program of gene expression is a hallmark of cancer that initiates and propagates tumorigenesis. Altered DNA methylation, histone modifications and ncRNAs expression are a feature of cancer cells. The dynamic epigenetic changes during oncogenic transformation are related to tumor heterogeneity, unlimited self-renewal and multi-lineage differentiation. This stem cell-like state or the aberrant reprogramming of cancer stem cells is the major challenge in treatment and drug resistance. Given the reversible nature of epigenetic modifications, the ability to restore the cancer epigenome through the inhibition of the epigenetic modifiers is a promising therapy for cancer treatment, either as a monotherapy or in combination with other anticancer therapies, including immunotherapies. Herein, we highlighted the main epigenetic alterations, their potential as a biomarker for early diagnosis and the epigenetic therapies approved for cancer treatment.

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Fifty years of clinical and research studies for childhood renal tumors within the International Society of Pediatric Oncology (SIOP)

Cited by 16 publications

References 50 publications

International Comparisons of Clinical Demographics and Outcomes in the International Society of Pediatric Oncology Wilms Tumor 2001 Trial and Study

International Comparisons of Clinical Demographics and Outcomes in the International Society of Pediatric Oncology Wilms Tumor 2001 Trial and Study

Similarities and controversies in imaging of pediatric renal tumors: A SIOP‐RTSG and COG collaboration

Epigenetic reprogramming in cancer: From diagnosis to treatment

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