Table 6 Children’s Oncology Group 2024 favourable-histology Wilms tumour treatment stratification

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

Stage	Age	Molecular Features	Lymph-node positive	Lung-nodule response	Extrapulmonary metastases	Post-chemotherapy histology	Other	AREN2231 final stratum assignment
I	Any	Normal	NA	NA	NA	NA	Epithelial histology AND no Wilms tumour-predisposing condition^a	Nephrectomy only
I	<4 years	Normal	NA	NA	NA	NA	No Wilms tumour-predisposing condition^a	Nephrectomy only
I	Any	Combined LOH, 1q gain OR 11p15 LOH	NA	NA	NA	NA	NA	EE-4A
I	>4	Any	NA	NA	NA	NA	NA	EE-4A
II	Any	Normal	NA	NA	NA	NA	NA	EE-4A
II	Any	Isolated LOH	NA	NA	NA	NA	NA	EE-4A
III	Any	Normal	Any	NA	NA	NA or low risk or intermediate risk	NA	EE-4A ± radiotherapy^b,c
III	Any	Isolated LOH	No	NA	NA	NA or low risk or intermediate risk	NA	EE-4A ± radiotherapy^b,c
II	Any	Combined LOH or 1q gain	NA	NA	NA	NA	NA	DD-4A vs VIVA
IV	Any	Normal	Any	RCR	No	NA or low risk or intermediate risk	NA	DD-4A ± radiotherapy^b
IV	Any	Isolated LOH	No	RCR	No	NA or low risk or intermediate risk	NA	DD-4A ± radiotherapy^b
III	Any	Combined LOH or 1q gain	Any	NA	NA	NA or low risk or intermediate risk	NA	Regimen M vs MVI + RT
III	Any	Isolated LOH	Yes	NA	NA	NA or low risk or intermediate risk	NA	Regimen M vs MVI^d + radiotherapy
IV	Any	Combined LOH or 1q gain	Any	Any	No	NA or low risk or intermediate risk	NA	Regimen M vs MVI + radiotherapy
IV	Any	Isolated LOH	Yes	Any	No	NA or low risk or intermediate risk	NA	Regimen M vs MVI^d + radiotherapy
IV	Any	Any	Any	SIR	No	NA or low risk or intermediate risk	NA	Regimen M vs MVI + radiotherapy
IV	Any	Any	Any	Any	Yes	NA or low risk or intermediate risk	NA	Regimen M vs MVI + radiotherapy
III	Any	Any	Any	NA	NA	High risk	NA	Regimen UH-3 + radiotherapy
IV	Any	Any	Any	Any	Any	High risk	NA	Regimen UH-3 + radiotherapy

Combined LOH, LOH of both 1p and 16q; Isolated LOH, LOH of either 1p or 16q; LOH, loss of heterozygosity; NA, not applicable; Normal, absence of isolated LOH, combined LOH or 1q gain — LOH of other loci only relevant if specified; RCR, rapid complete response; SIR, slow incomplete response. ^aWilms tumour-predisposing conditions include (but are not limited to) genetic syndromes such as idiopathic hemihypertrophy or isolated lateralized overgrowth, Beckwith–Wiedemann syndrome, Denys–Drash syndrome, WAGR (Wilms tumour, aniridia, genitourinary anomalies, range of developmental delays) syndrome, trisomy 18, Simpson–Golabi–Behmel syndrome, Bohring–Opitz syndrome and unilateral Wilms tumour with multifocal tumours or contralateral nephrogenic rests. ^bAbdominal radiotherapy (flank or whole abdominal irradiation) for all patients with local stage III disease except those with nonmalignant biology and low-risk post-chemotherapy histology. ^cA subset of patients who undergo delayed nephrectomy after 3–4 cycles of DD-4A will not be eligible for de-escalation to EE-4A or omission of radiotherapy, and will receive DD-4A with abdominal (flank or whole abdominal irradiation) radiotherapy. ^dA subset of patients with positive lymph nodes not discovered until nephrectomy after 3–4 cycles of chemotherapy will not be eligible for regimen M versus MVI randomization and will be directly assigned to regimen M.

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Table 6 Children’s Oncology Group 2024 favourable-histology Wilms tumour treatment stratification

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