Wilms Tumour
Wilms tumour is the most common kidney cancer of childhood and one of the clearest successes of collaborative paediatric oncology, with outcomes for most children now very good. Histology and stage drive treatment discussion.
Disease categories
Major disease categories
- Favourable histology Wilms tumour
- Anaplastic (unfavourable histology) Wilms tumour
- Bilateral Wilms tumour
- Nephrogenic rests
Molecular
Molecular considerations
WT1, CTNNB1 and WTX alterations are described. Loss of heterozygosity at 1p and 16q, and 1q gain, carry prognostic weight. TP53 alteration is associated with anaplastic histology.
Markers commonly discussed in wilms tumour
- WT1
- LOH 1p / 16q
- 1q gain
- TP53
- Histology (anaplasia)
Not exhaustive, and not every marker is assessed in every patient. Which of these is relevant depends on the individual case and should be discussed with the treating team.
Testing
Genomic testing considerations
Loss of heterozygosity assessment is used in risk stratification in some protocols.
Germline
Germline considerations
Germline assessment is discussed particularly in bilateral disease, young age at diagnosis, or where syndromic features suggest WAGR, Denys-Drash or Beckwith-Wiedemann syndrome.
Molecular monitoring
ctDNA and liquid biopsy considerations
Investigational.
Single-cell & computation
Single-cell and algorithmic approaches
Paediatric tumours are frequently driven by a single fusion or copy-number event on a quiet mutational background, which makes single-cell analysis unusually informative about developmental state rather than mutational burden. Single-cell RNA sequencing is used to place a tumour along a developmental trajectory. Algorithm work includes fusion detection, methylation classification, and analysis designed for the small cohort sizes that paediatric rarity imposes.
Decisions
Treatment decision questions
Questions worth raising with a treating team when a decision is being made:
- Is the histology documented, including the presence or absence of anaplasia?
- Has bilateral disease been excluded on imaging?
- Have syndromic associations been assessed?
Resistance
Resistance and recurrence questions
- Has the histology been reviewed at relapse?
Trials
Clinical trial considerations
Paediatric trials run through collaborative specialist networks.
When a precision oncology review may help
A review is most useful where the standard pathway has been exhausted or where the picture is more complicated than a protocol accounts for — rare presentations, treatment resistance, complex or apparently contradictory molecular findings, or a decision between several defensible options.
The output is a structured analysis and a set of questions for the treating team. It is not treatment, and it does not replace the treating oncologist.