The UMBRELLA SIOP-RTSG 2016 Wilms tumour pathology and molecular biology protocol

Gordan M Vujanić, Manfred Gessler, Ariadne H A G Ooms, Paola Collini, Aurore Coulomb-l'Hermine, Ellen D'Hooghe, Ronald R de Krijger, Daniela Perotti, Kathy Pritchard-Jones, Christian Vokuhl, Marry M van den Heuvel-Eibrink, Norbert Graf, International Society of Paediatric Oncology–Renal Tumour Study Group (SIOP–RTSG), Gordan M Vujanić, Manfred Gessler, Ariadne H A G Ooms, Paola Collini, Aurore Coulomb-l'Hermine, Ellen D'Hooghe, Ronald R de Krijger, Daniela Perotti, Kathy Pritchard-Jones, Christian Vokuhl, Marry M van den Heuvel-Eibrink, Norbert Graf, International Society of Paediatric Oncology–Renal Tumour Study Group (SIOP–RTSG)

Abstract

On the basis of the results of previous national and international trials and studies, the Renal Tumour Study Group of the International Society of Paediatric Oncology (SIOP-RTSG) has developed a new study protocol for paediatric renal tumours: the UMBRELLA SIOP-RTSG 2016 protocol (the UMBRELLA protocol). Currently, the overall outcomes of patients with Wilms tumour are excellent, but subgroups with poor prognosis and increased relapse rates still exist. The identification of these subgroups is of utmost importance to improve treatment stratification, which might lead to reduction of the direct and late effects of chemotherapy. The UMBRELLA protocol aims to validate new prognostic factors, such as blastemal tumour volume and molecular markers, to further improve outcome. To achieve this aim, large, international, high-quality databases are needed, which dictate optimization and international harmonization of specimen handling and comprehensive sampling of biological material, refine definitions and improve logistics for expert review. To promote broad implementation of the UMBRELLA protocol, the updated SIOP-RTSG pathology and molecular biology protocol for Wilms tumours has been outlined, which is a consensus from the SIOP-RTSG pathology panel.

Conflict of interest statement

The authors declare no competing interests.

Figures

Fig. 1. Flow diagram of rapid central…
Fig. 1. Flow diagram of rapid central pathology review.
After nephrectomy, the institutional pathologist biobanks the specimen and examines it according to the UMBRELLA protocol, producing a report. The institutional pathologist then sends the complete set of slides for rapid central pathology review to their regional or national panel. The chair of the regional or national pathology panel promptly undertakes central pathology review and sends back their results to the institutional pathologist within 1 week. The International Society of Paediatric Oncology (SIOP) pathology panel reviews cases from the regional and national pathology panels restrospectively.
Fig. 2. Biobanking and biomarkers in the…
Fig. 2. Biobanking and biomarkers in the UMBRELLA protocol.
Tissue, blood and urine samples are collected locally and included in national biobanks that are virtually connected. These materials are primarily used to answer UMBRELLA protocol questions. Any surplus materials will be made available for ancillary and focused studies.

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