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Nat Rev Urol. 2018 Nov;15(11):693-701. doi: 10.1038/s41585-018-0100-3.

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

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Department of Pathology, Sidra Medicine, Doha, Qatar.
Theodor-Boveri-Institute/Biocenter, Developmental Biochemistry, Wuerzburg University, Wuerzburg, Germany.
Comprehensive Cancer Center Mainfranken, Wuerzburg University, Wuerzburg, Germany.
Princess Maxima Centre for Pediatric Oncology, Utrecht, Netherlands.
Department of Diagnostic Pathology and Laboratory Medicine, Fondazione IRCCS Istituto Nazionale dei Tumori, Milan, Italy.
Sorbonne Université, Department of Pathology, Hopitaux Universitaires Est Parisien, Paris, France.
Department of Pathology, Oslo University Hospital, Rikshospitalet, Oslo, Norway.
Molecular Bases of Genetic Risk and Genetic Testing Unit, Department of Preventive and Predictive Medicine, Fondazione IRCCS Istituto Nazionale dei Tumori, Milan, Italy.
Great Ormond Street Institute of Child Health, University College London, London, UK.
Kiel Paediatric Tumour Registry, Department of Paediatric Pathology, University Hospital of Kiel, Kiel, Germany.
Department of Paediatric Oncology & Haematology, Saarland University, Homburg, Germany.


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.

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