Table 1 First-generation Children’s Oncology Group favourable-histology Wilms tumour risk stratification

From: Updated favourable-histology Wilms tumour risk stratification: rationale for future Children’s Oncology Group clinical trials

Stage

Age

Tumour weight

Molecular features

Lung nodule response

EPM

Risk groupa

I

<2 years

<550 g

Any

NA

NA

Very low risk

I

<2 years

>550 g

Normal

NA

NA

Low risk

I

>2 years

Any

Normal

NA

NA

Low risk

II

Any

Any

Normal

NA

NA

Low risk

I

Any

Any

Combined LOH

NA

NA

Standard risk

II

Any

Any

Combined LOH

NA

NA

Standard risk

III

Any

Any

Normal

NA

NA

Standard risk

IV

Any

Any

Normal

RCR

No

Standard risk

III

Any

Any

Combined LOH

NA

NA

Higher risk

IV

Any

Any

Combined LOH

Any

No

Higher risk

IV

Any

Any

Any

SIR

No

Higher risk

IV

Any

Any

Any

Any

Yes

Higher risk

  1. Combined LOH, LOH of both 1p and 16q; EPM, extrapulmonary metastases; LOH, loss of heterozygosity; NA, not applicable; Normal, absence of combined LOH; RCR, rapid complete response; SIR, slow incomplete response. aRisk group assignment does not necessarily indicate the optimal treatment. Treatment changes in AREN0532 or AREN0533 did not improve outcomes for all patients, yet these risk group categories still represent relative differences in outcomes for these groups of patients. Adapted with permission from ref. 16, Wiley.