Informations générales (source: ClinicalTrials.gov)
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 (UMBRELLA)
Interventional
N/A
Assistance Publique Hopitaux De Marseille (Voir sur ClinicalTrials)
août 2019
août 2026
05 avril 2025
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 10,000 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.
Etablissements
| Les établissements d'Île-de-France ayant mis à jour leurs données Origine et niveau de fiabilité des données | |||||
|---|---|---|---|---|---|
| CLCC INSTITUT CURIE | 10/04/2025 13:12:16 | Contact (sur clinicalTrials) | |||
| CLCC INSTITUT GUSTAVE ROUSSY | Claudia PASQUALINI | 23/05/2024 13:27:01 | Contacter | ||
| Les établissements hors Île-de-France dont les données sont issues de ClinicalTrials.gov Origine et niveau de fiabilité des données | |||||
| Service d'Hématologie-Oncologie Pédiatrique - APHM - 13354 - Marseille - France | Arnauld VERSCHUUR | Contact (sur clinicalTrials) | |||
Critères
Tous
Inclusion Criteria:
- Children, adolescents or young adults (up to and including 30 years) with primary or
relapsed renal tumor diagnosed at a participating SIOP-RTSG center
- Subject agreeing to participate
- Children, adolescents or young adults (up to and including 30 years) with primary or
relapsed renal tumor diagnosed at a participating SIOP-RTSG center
- Subject agreeing to participate
- Absence of informed consent