Spreafico, F;
Fernandez, CV;
Brok, J;
Nakata, K;
Vujanic, G;
Geller, JI;
Gessler, M;
... Pritchard-Jones, K; + view all
(2021)
Wilms tumour.
Nature Reviews Disease Primers
, 7
, Article 75. 10.1038/s41572-021-00308-8.
Preview |
Text (Article)
Pritchard-Jones_Wilms tumour_Primer for UCL upload.pdf - Accepted Version Download (574kB) | Preview |
Preview |
Text (Supplementary information)
Pritchard-Jones_Supplementary information. Nature Primer.pdf - Accepted Version Download (142kB) | Preview |
![[thumbnail of Figures]](https://discovery-pp.ucl.ac.uk/style/images/fileicons/slideshow.png) |
Slideshow (Figures)
Figures_Primer.pptx - Accepted Version Download (2MB) |
Abstract
Wilms tumour (WT) is a childhood embryonal tumour that is paradigmatic of the intersection between disrupted organogenesis and tumorigenesis. Many WT genes play a critical (non-redundant) role in early nephrogenesis. Improving patient outcomes requires advances in understanding and targeting of the multiple genes and cellular control pathways now identified as active in WT development. Decades of clinical and basic research have helped to gradually optimize clinical care. Curative therapy is achievable in 90% of affected children, even those with disseminated disease, yet survival disparities within and between countries exist and deserve commitment to change. Updated epidemiological studies have also provided novel insights into global incidence variations. Introduction of biology-driven approaches to risk stratification and new drug development has been slower in WT than in other childhood tumours. Current prognostic classification for children with WT is grounded in clinical and pathological findings and in dedicated protocols on molecular alterations. Treatment includes conventional cytotoxic chemotherapy and surgery, and radiation therapy in some cases. Advanced imaging to capture tumour composition, optimizing irradiation techniques to reduce target volumes, and evaluation of newer surgical procedures are key areas for future research.
| Type: | Article |
|---|---|
| Title: | Wilms tumour |
| Open access status: | An open access version is available from UCL Discovery |
| DOI: | 10.1038/s41572-021-00308-8 |
| Publisher version: | http://dx.doi.org/10.1038/s41572-021-00308-8 |
| Language: | English |
| Additional information: | This version is the author accepted manuscript. For information on re-use, please refer to the publisher’s terms and conditions. |
| Keywords: | CHILDRENS ONCOLOGY GROUP, STAGE RENAL-DISEASE, INTERNAL TANDEM DUPLICATIONS, 2ND MALIGNANT NEOPLASMS, CLEAR-CELL SARCOMA, AT-RISK CHILDREN, SIOP WT 2001, CHILDHOOD-CANCER, PROGNOSTIC-FACTORS, NEPHROGENIC RESTS |
| UCL classification: | UCL UCL > Provost and Vice Provost Offices > School of Life and Medical Sciences UCL > Provost and Vice Provost Offices > School of Life and Medical Sciences > Faculty of Population Health Sciences > UCL GOS Institute of Child Health UCL > Provost and Vice Provost Offices > School of Life and Medical Sciences > Faculty of Population Health Sciences > UCL GOS Institute of Child Health > Developmental Biology and Cancer Dept |
| URI: | https://discovery-pp.ucl.ac.uk/id/eprint/10139854 |
Archive Staff Only
 |
View Item |
