Paediatric kidney tumours are generally associated with a favourable survival rate. Most children are diagnosed with Wilms tumour, which has a 90% long-term survival rate with conventional front-line and salvage therapies. However, treatments and outcomes of children with relapsed non-Wilms tumours, such as malignant rhabdoid tumour of the kidney, renal-cell carcinoma (including renal medullary carcinoma), clear-cell sarcoma of the kidney, anaplastic sarcoma of the kidney and congenital mesoblastic nephroma are not well defined. Several of these non-Wilms tumours include unfavourable prognostic subtypes. Currently available data on non-Wilms tumours support advancing studies that pivot from conventional strategies towards disease-specific, biologically driven novel treatments to improve outcomes for each of these rare childhood kidney tumours.

Relapsed rhabdoid tumours and other non-nephroblastoma childhood and adolescent kidney tumours: perspectives from the HARMONICA collaboration

Quarello, Paola;Perotti, Daniela;
2026-01-01

Abstract

Paediatric kidney tumours are generally associated with a favourable survival rate. Most children are diagnosed with Wilms tumour, which has a 90% long-term survival rate with conventional front-line and salvage therapies. However, treatments and outcomes of children with relapsed non-Wilms tumours, such as malignant rhabdoid tumour of the kidney, renal-cell carcinoma (including renal medullary carcinoma), clear-cell sarcoma of the kidney, anaplastic sarcoma of the kidney and congenital mesoblastic nephroma are not well defined. Several of these non-Wilms tumours include unfavourable prognostic subtypes. Currently available data on non-Wilms tumours support advancing studies that pivot from conventional strategies towards disease-specific, biologically driven novel treatments to improve outcomes for each of these rare childhood kidney tumours.
2026
0
6
Ortiz, Michael V.; Wens, Francis S.P.L.; Hong, Andrew L.; Quarello, Paola; Verschuur, Arnauld C.; Tracy, Elisabeth T.; Daw, Najat C.; Dome, Jeffrey S....espandi
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Utilizza questo identificativo per citare o creare un link a questo documento: https://hdl.handle.net/2318/2143297
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