Table 5 Levels of evidence for favourable-histology Wilms tumour risk-stratification features

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

Level of evidence117

Feature

Part of updated COG risk stratification?

I

Stage

Yes

Histology

Yes

LOH of 1p and 16q

Yes

Lung metastatic response to chemotherapy

Yes

II

Lymph node sampling

Yes

Age

Yes

Tumour nephrectomy weight

No

Extra pulmonary metastasisa

Yes

11p15 status in patients with very-low-risk disease

Yes

Epithelial-predominant histology

Yes

1q gain

Yes

Lymph node involvement with isolated LOH 1p/16q

Yes

Post-chemotherapy blastemal-predominant histology

Yes

Peritoneal metastases

Yesb

Partial nephrectomy for unilateral Wilms tumour

No

Unilateral multifocal tumours

No

III

Tumour involving adrenal gland

Nob

Inferior vena cava thrombus

Nob

IV

Extrarenal Wilms tumour

No

V

Malignant pleural effusions

Yesb

Tumour pulmonary emboli

Yesb

Extra-abdominal lymph nodes not pathologically sampled

Nob

Malignant ascites (peritoneal fluid)

No

Contralateral nephrogenic rests

No

Tumour in renal collecting system

Nob

Genetic predisposition to Wilms tumour

Yesc

  1. Data collated from refs. 5,7,8,9,11,12,14,17,22,23,24,27,28,34,41,53,54,60,61,66,67,69,71,79,80,81,83,84,85,86,88,100,101,102,105,106,108,109. Level I is a high-quality prospective cohort study with adequate power or a systematic review of these studies. Level II is a reduced-quality prospective cohort study, retrospective cohort study, untreated healthy participants from a randomized control trial or a systematic review of these studies. Level III is a case–control study or a systematic review of these studies. Level IV is case series. Level V is an expert opinion, a case report or clinical example, or evidence based on physiology, bench research or ‘first principles’. LOH, loss of heterozygosity. aSome features considered extrapulmonary metastasis have reduced-quality evidence. bFeature is incorporated into stage, but not independently factored into risk stratification. cPatients with genetic predisposition to Wilms tumour are excluded from treatment with nephrectomy only.