Ignite Creation Date:
2024-05-06 @ 1:21 PM
Last Modification Date:
2024-10-26 @ 1:13 PM
Study NCT ID:
NCT04005820
Status:
NOT_YET_RECRUITING
Last Update Posted:
2019-07-02
First Post:
2019-06-12
Brief Title:
Impact of Tumour 1q Gain in French Pediatric and Young Adult Patients With Renal Tumours
Sponsor:
Assistance Publique Hopitaux De Marseille
Organization:
Assistance Publique Hopitaux De Marseille
Study Overview
Official Title:
Treatment for French Pediatric Patients With Renal Tumours According to the International SIOP Renal Tumour Study Group RTSG 2016 UMBRELLA Protocol Including Analysis of Tumour Chromosome 1q Gain and Central Radiology Review
Status:
NOT_YET_RECRUITING
Status Verified Date:
2019-06
Last Known Status:
None
Delayed Posting:
No
If Stopped, Why?:
Not Stopped
Has Expanded Access:
False
If Expanded Access, NCT#:
N/A
Has Expanded Access, NCT# Status:
N/A
Acronym:
UMBRELLA
Brief Summary:
French patients with nephroblastoma Wilms tumour WT have been treated for 40 years according to International Society of Paediatric Oncology SIOP protocols with currently 267 centres across 28 countries collaborating internationally within the SIOP Renal Tumour Study Group RTSG
Over the last decades more than 10000 children have been prospectively enrolled in SIOP WT studies and trials This has resulted in more standardised diagnostic procedures improved risk stratification and adjusted treatment recommendations for most renal tumours The treatment of patients with renal tumours according to SIOP protocols include preoperative chemotherapy surgery tumour-nephrectomy node-picking metastasectomy followed by risk- and stage-based postoperative chemotherapy radiotherapy Central pathology review is nowadays routinely performed in order to prevent misclassification of stage and histology risk group
The current SIOP 2001 protocol has come to an end with as major achievement the scientific proof of omitting doxorubicin in stage II and III patients with as a consequence less risk of sequelae Moreover in the SIOP 2001 protocol several tumour biological aspects have been assessed that seem to interfere with outcome chromosomal gain of 1q or loss of 1p and 16q blastemal residual volume Chromosomal 1q gain is considered to be present in 25-35 of patients with nephroblastoma with a negative impact on event-free survival EFS in retrospective analyses These biological aspects will be studied prospectively as a primary objective in the new SIOP RTSG 2016 UMBRELLA protocol that integrates diagnostics treatment and follow-up guidelines as well as several research projects
The main mission of the International Society of Paediatric Oncology SIOP Renal Tumour Study Group RTSG is to increase survival and to reduce acute treatment toxicity and late effects in all children diagnosed with any renal tumour In this context SIOP RTSG is aiming to offer all these patients the same standardized high quality diagnostics and treatment independent of the tumour type The new SIOP RTSG 2016 integrated diagnostic and research UMBRELLA protocol serves as an entry for including all children with a renal tumour in the SIOP-RTSG centers including prospective biomarker analyses Subsequently treatment is recommended according to the SIOP RTSG 2016 UMBRELLA treatment guidelines which provides treatment strategies for all patients with Wilms tumour WT and other renal tumours Central radiology review CRR has been proposed as a novel tool within the diagnostic UMBRELLA protocol in order to optimize the diagnostics and hence the treatment
The definition of metastatic disease in WT remains difficult since pulmonary nodules may not always be of malignant origin The differential diagnosis of a pulmonary lesion seen in a child with WT is broad In addition to malignancy it includes atelectasis fibrosis pneumonitis subpleural lymph nodes and other infectious or inflammatory lesions In addition the issue of CT-only nodules in WT and adequate treatment needs to be solved In previous protocols the treatment strategy was based on the diagnosis of pulmonary metastases 92 of all metastases by conventional pulmonary X-ray Patients with CT-only nodules nodules not visible on conventional X-ray were supposed to be treated as having localized WT However retrospective analyses of SIOP series Smets et al showed that patients with CT-only nodules had a less favourable prognosis as compared to patients with truly localized disease with a 12 difference in three-year event-free survival
The diagnostics of bilateral renal tumours stage V often is complicated since it may be difficult to distinguish true WT from nephroblastomatosis nephrogenic rests a pre malignant renal multifocal anomaly which may respond to preoperative chemotherapy An optimal multi-disciplinary sequential diagnostic procedure is required in order to propose the best adapted therapeutic approach to preserve sufficient renal tissue
Over the last decades more than 10000 children have been prospectively enrolled in SIOP WT studies and trials This has resulted in more standardised diagnostic procedures improved risk stratification and adjusted treatment recommendations for most renal tumours The treatment of patients with renal tumours according to SIOP protocols include preoperative chemotherapy surgery tumour-nephrectomy node-picking metastasectomy followed by risk- and stage-based postoperative chemotherapy radiotherapy Central pathology review is nowadays routinely performed in order to prevent misclassification of stage and histology risk group
The current SIOP 2001 protocol has come to an end with as major achievement the scientific proof of omitting doxorubicin in stage II and III patients with as a consequence less risk of sequelae Moreover in the SIOP 2001 protocol several tumour biological aspects have been assessed that seem to interfere with outcome chromosomal gain of 1q or loss of 1p and 16q blastemal residual volume Chromosomal 1q gain is considered to be present in 25-35 of patients with nephroblastoma with a negative impact on event-free survival EFS in retrospective analyses These biological aspects will be studied prospectively as a primary objective in the new SIOP RTSG 2016 UMBRELLA protocol that integrates diagnostics treatment and follow-up guidelines as well as several research projects
The main mission of the International Society of Paediatric Oncology SIOP Renal Tumour Study Group RTSG is to increase survival and to reduce acute treatment toxicity and late effects in all children diagnosed with any renal tumour In this context SIOP RTSG is aiming to offer all these patients the same standardized high quality diagnostics and treatment independent of the tumour type The new SIOP RTSG 2016 integrated diagnostic and research UMBRELLA protocol serves as an entry for including all children with a renal tumour in the SIOP-RTSG centers including prospective biomarker analyses Subsequently treatment is recommended according to the SIOP RTSG 2016 UMBRELLA treatment guidelines which provides treatment strategies for all patients with Wilms tumour WT and other renal tumours Central radiology review CRR has been proposed as a novel tool within the diagnostic UMBRELLA protocol in order to optimize the diagnostics and hence the treatment
The definition of metastatic disease in WT remains difficult since pulmonary nodules may not always be of malignant origin The differential diagnosis of a pulmonary lesion seen in a child with WT is broad In addition to malignancy it includes atelectasis fibrosis pneumonitis subpleural lymph nodes and other infectious or inflammatory lesions In addition the issue of CT-only nodules in WT and adequate treatment needs to be solved In previous protocols the treatment strategy was based on the diagnosis of pulmonary metastases 92 of all metastases by conventional pulmonary X-ray Patients with CT-only nodules nodules not visible on conventional X-ray were supposed to be treated as having localized WT However retrospective analyses of SIOP series Smets et al showed that patients with CT-only nodules had a less favourable prognosis as compared to patients with truly localized disease with a 12 difference in three-year event-free survival
The diagnostics of bilateral renal tumours stage V often is complicated since it may be difficult to distinguish true WT from nephroblastomatosis nephrogenic rests a pre malignant renal multifocal anomaly which may respond to preoperative chemotherapy An optimal multi-disciplinary sequential diagnostic procedure is required in order to propose the best adapted therapeutic approach to preserve sufficient renal tissue
Detailed Description:
None
Study Oversight
Has Oversight DMC:
None
Is a FDA Regulated Drug?:
False
Is a FDA Regulated Device?:
False
Is an Unapproved Device?:
None
Is a PPSD?:
None
Is a US Export?:
None
Is an FDA AA801 Violation?:
None