Treatment algorithm

Please note that formulations/routes and doses may differ between drug names and brands, drug formularies, or locations. Treatment recommendations are specific to patient groups: see disclaimer

ACUTE

COG criteria

1st line – surgery

Back
ACUTE
|
COG criteria
|
favourable histology: stage I
1st line – 

surgery

Upfront nephrectomy using a transperitoneal approach is recommended for patients with unilateral renal masses who do not have a known Wilms' tumour predisposition.[3][40]

Surgeon should avoid tumour spillage or incomplete removal. Selective sampling of lymph nodes should also be performed.[72]

Palpation of renal vein and inferior vena cava identifies any tumour thrombi (usually free-floating) and may allow for careful removal of thrombus.[40]

Any suspicious metastatic lesion (hepatic or intra-abdominal) should be either biopsied or resected.[40]

Biopsy remains controversial, but an open biopsy with a minimum of 10-12 cores is required in the Children's Oncology Group (COG) protocol if a tumour is unresectable.[3][40]

Consider – postoperative chemotherapy

Back
ACUTE
|
COG criteria
|
favourable histology: stage I
Consider – 

postoperative chemotherapy

Additional treatment recommended for SOME patients in selected patient group

Chemotherapy regimen depends on staging and histology of tumour.

COG very-low risk patients (<2 years of age, <550 g tumour weight, stage I, any loss of heterozygosity [LOH] status): observation alone and a trial-protocol directed approach to relapse (COG study #AREN0532) or vincristine and dactinomycin (EE-4A regimen) if not being treated on a study.[83][84]

COG low-risk patients (any age or tumour weight, stage I or II, but no LOH at 1p and 16q): EE-4A regimen.[83]

COG standard-risk patients (stage I tumours ≥550 g with LOH at 1p and 16q): vincristine, dactinomycin, and doxorubicin (DD-4A regimen).[83][85]

See local specialist protocol for dosing guidelines.

Primary options

EE-4A regimen

vincristine

and

dactinomycin

OR

DD-4A regimen

vincristine

and

dactinomycin

and

doxorubicin

1st line – surgery

Back
ACUTE
|
COG criteria
|
favourable histology: stage II
1st line – 

surgery

Upfront nephrectomy using a transperitoneal approach is recommended for patients with unilateral renal masses who do not have a known Wilms' tumour predisposition.[3][40]

Surgeon should avoid tumour spillage or incomplete removal. Selective sampling of lymph nodes should also be performed.[72]

Palpation of renal vein and inferior vena cava identifies any tumour thrombi (usually free-floating) and may allow for careful removal of thrombus.[40]

Any suspicious metastatic lesion (hepatic or intra-abdominal) should be either biopsied or resected.[40]

Biopsy remains controversial, but an open biopsy with a minimum of 10-12 cores is required in the Children's Oncology Group (COG) protocol if a tumour is unresectable.[3][40]

Plus – postoperative chemotherapy

Back
ACUTE
|
COG criteria
|
favourable histology: stage II
Plus – 

postoperative chemotherapy

Treatment recommended for ALL patients in selected patient group

Chemotherapy regimen depends on staging and histology of tumour.

COG low-risk patients (any age or tumour weight, stage I or II, but no loss of heterozygosity [LOH] at 1p and 16q): vincristine and dactinomycin (EE-4A regimen).[83]

COG standard-risk patients (any weight stage II tumours with LOH at 1p and 16q): vincristine, dactinomycin, and doxorubicin (DD-4A regimen).

See local specialist protocol for dosing guidelines.

Primary options

EE-4A regimen

vincristine

and

dactinomycin

OR

DD-4A regimen

vincristine

and

dactinomycin

and

doxorubicin

1st line – surgery

Back
ACUTE
|
COG criteria
|
favourable histology: stage III
1st line – 

surgery

Upfront nephrectomy using a transperitoneal approach is recommended for patients with unilateral renal masses who do not have a known Wilms' tumour predisposition.[3][40]

Surgeon should avoid tumour spillage or incomplete removal. Selective sampling of lymph nodes should also be performed.[72]

Palpation of renal vein and inferior vena cava identifies any tumour thrombi (usually free-floating) and may allow for careful removal of thrombus.[40]

Any suspicious metastatic lesion (hepatic or intra-abdominal) should be either biopsied or resected.[40]

Biopsy remains controversial, but an open biopsy with a minimum of 10-12 cores is required in the Children's Oncology Group (COG) protocol if a tumour is unresectable.[3][40]

Plus – postoperative chemotherapy

Back
ACUTE
|
COG criteria
|
favourable histology: stage III
Plus – 

postoperative chemotherapy

Treatment recommended for ALL patients in selected patient group

Chemotherapy regimen depends on staging and histology of tumour.

COG standard-risk patients (stage III with no loss of heterozygosity [LOH] at 1p and 16q): vincristine, dactinomycin, and doxorubicin (DD-4A regimen).[86]

COG high-risk patients (stage III with LOH at 1p and 16q): DD-4A regimen for 6 weeks, and then switched to regimen M (vincristine, dactinomycin, doxorubicin, cyclophosphamide, and etoposide).[83][85]

See local specialist protocol for dosing guidelines.

Primary options

DD-4A regimen

vincristine

and

dactinomycin

and

doxorubicin

OR

Regimen M

vincristine

and

dactinomycin

and

doxorubicin

and

cyclophosphamide

and

etoposide

Plus – radiotherapy

Back
ACUTE
|
COG criteria
|
favourable histology: stage III
Plus – 

radiotherapy

Treatment recommended for ALL patients in selected patient group

All stage III tumours receive either flank irradiation or whole abdomen irradiation.[83][85]

1st line – surgery

Back
ACUTE
|
COG criteria
|
favourable histology: stage IV
1st line – 

surgery

Upfront nephrectomy using a transperitoneal approach is recommended for patients with unilateral renal masses who do not have a known Wilms' tumour predisposition.[3][40]

Surgeon should avoid tumour spillage or incomplete removal. Selective sampling of lymph nodes should also be performed.[72]

Palpation of renal vein and inferior vena cava identifies any tumour thrombi (usually free-floating) and may allow for careful removal of thrombus.[40]

Any suspicious metastatic lesion (hepatic or intra-abdominal) should be either biopsied or resected.[40]

Biopsy remains controversial, but an open biopsy with a minimum of 10-12 cores is required in the Children's Oncology Group (COG) protocol if a tumour is unresectable.[3][40]

Plus – postoperative chemotherapy

Back
ACUTE
|
COG criteria
|
favourable histology: stage IV
Plus – 

postoperative chemotherapy

Treatment recommended for ALL patients in selected patient group

Chemotherapy regimen depends on staging and histology of tumour.

COG standard-risk (stage IV with no loss of heterozygosity [LOH] at 1p and 16q): vincristine, dactinomycin, and doxorubicin (DD-4A regimen).[82][83]​​ Patients with pulmonary metastases with a complete response at week 6 continue receiving DD-4A regimen chemotherapy.[82] Patients with an incomplete/slow response of pulmonary metastatic lesions at week 6 are switched to chemotherapy with vincristine, dactinomycin, doxorubicin, cyclophosphamide, and etoposide (regimen M).[82]

COG high-risk (stage IV with LOH at 1p and 16q): vincristine, dactinomycin, doxorubicin, cyclophosphamide, and etoposide (regimen M).[83][85]

See local specialist protocol for dosing guidelines.

Primary options

DD-4A regimen

vincristine

and

dactinomycin

and

doxorubicin

OR

Regimen M

vincristine

and

dactinomycin

and

doxorubicin

and

cyclophosphamide

and

etoposide

Plus – radiotherapy

Back
ACUTE
|
COG criteria
|
favourable histology: stage IV
Plus – 

radiotherapy

Treatment recommended for ALL patients in selected patient group

COG standard-risk (no LOH): abdominal/flank irradiation; patients with an incomplete/slow response of pulmonary metastatic lesions at week 6 receive whole lung irradiation.[82][83]​​

COG high-risk (LOH present): abdominal/flank irradiation and whole lung irradiation.[83][85]

1st line – preoperative chemotherapy

Back
ACUTE
|
COG criteria
|
favourable histology: stage V
1st line – 

preoperative chemotherapy

In patients with bilateral Wilms' tumour or bilaterally predisposed unilateral Wilms' tumour, preoperative chemotherapy is followed by nephron-sparing surgery and modified postoperative chemotherapy based on histological response.[83][87][88]

The goal of preoperative chemotherapy is to shrink the tumour to allow maximum preservation of renal parenchyma.[88]

See local specialist protocol for regimen and dosing guidelines.

Plus – surgery

Back
ACUTE
|
COG criteria
|
favourable histology: stage V
Plus – 

surgery

Treatment recommended for ALL patients in selected patient group

Radical nephrectomies can potentially be avoided and nephron-sparing surgery (e.g., unilateral total nephrectomy with contralateral partial nephrectomy, bilateral partial nephrectomy, unilateral total nephrectomy, and unilateral partial nephrectomy) used to preserve renal parenchyma and function.[87]

Partial nephrectomy may not be sufficient for high-risk patients due to a high incidence of recurrence.[89][90][91]

Plus – postoperative chemotherapy

Back
ACUTE
|
COG criteria
|
favourable histology: stage V
Plus – 

postoperative chemotherapy

Treatment recommended for ALL patients in selected patient group

Postoperative chemotherapy is based on histological response.[83][87][88]

Each side should be individually staged and treated according to the guidelines above.

See local specialist protocol for regimen and dosing guidelines.

Consider – renal transplant

Back
ACUTE
|
COG criteria
|
favourable histology: stage V
Consider – 

renal transplant

Additional treatment recommended for SOME patients in selected patient group

Rarely, disease is extensive bilaterally resulting in renal failure and need for a renal transplant.[92]

1st line – surgery

Back
ACUTE
|
COG criteria
|
anaplastic histology: stage I
1st line – 

surgery

Upfront nephrectomy using a transperitoneal approach is recommended for patients with unilateral renal masses who do not have a known Wilms' tumour predisposition.[3][40]

Surgeon should avoid tumour spillage or incomplete removal. Selective sampling of lymph nodes should also be performed.[72]

Palpation of renal vein and inferior vena cava identifies any tumour thrombi (usually free-floating) and may allow for careful removal of thrombus.[40]

Any suspicious metastatic lesion (hepatic or intra-abdominal) should be either biopsied or resected.[40]

Biopsy remains controversial, but an open biopsy with a minimum of 10-12 cores is required in the Children's Oncology Group (COG) protocol if a tumour is unresectable.[3][40]

Plus – postoperative chemotherapy

Back
ACUTE
|
COG criteria
|
anaplastic histology: stage I
Plus – 

postoperative chemotherapy

Treatment recommended for ALL patients in selected patient group

Stage I patients with either focal or diffuse anaplasia receive vincristine, dactinomycin, and doxorubicin (DD-4A regimen).[93]

See local specialist protocol for dosing guidelines.

Primary options

DD-4A regimen

vincristine

and

dactinomycin

and

doxorubicin

Plus – radiotherapy

Back
ACUTE
|
COG criteria
|
anaplastic histology: stage I
Plus – 

radiotherapy

Treatment recommended for ALL patients in selected patient group

Stage I patients with either focal or diffuse anaplasia receive flank irradiation.[93]

1st line – surgery

Back
ACUTE
|
COG criteria
|
anaplastic histology: stage II
1st line – 

surgery

Upfront nephrectomy using a transperitoneal approach is recommended for patients with unilateral renal masses who do not have a known Wilms' tumour predisposition.[3][40]

Surgeon should avoid tumour spillage or incomplete removal. Selective sampling of lymph nodes should also be performed.[72]

Palpation of renal vein and inferior vena cava identifies any tumour thrombi (usually free-floating) and may allow for careful removal of thrombus.[40]

Any suspicious metastatic lesion (hepatic or intra-abdominal) should be either biopsied or resected.[40]

Biopsy remains controversial, but an open biopsy with a minimum of 10-12 cores is required in the Children's Oncology Group (COG) protocol if a tumour is unresectable.[3][40]

Plus – postoperative chemotherapy

Back
ACUTE
|
COG criteria
|
anaplastic histology: stage II
Plus – 

postoperative chemotherapy

Treatment recommended for ALL patients in selected patient group

Treatment regimen depends on whether anaplasia is focal or diffuse.

Focal anaplasia: vincristine, dactinomycin, and doxorubicin (DD-4A regimen).[94]

Diffuse anaplasia: vincristine, doxorubicin, cyclophosphamide, carboplatin, and etoposide (revised UH-1 regimen).[83][95]

See local specialist protocol for dosing guidelines.

Primary options

DD-4A regimen

vincristine

and

dactinomycin

and

doxorubicin

OR

revised UH-1 regimen

vincristine

and

doxorubicin

and

cyclophosphamide

and

carboplatin

and

etoposide

Plus – radiotherapy

Back
ACUTE
|
COG criteria
|
anaplastic histology: stage II
Plus – 

radiotherapy

Treatment recommended for ALL patients in selected patient group

Patients with focal or diffuse anaplasia should receive flank irradiation.[83][94]

1st line – surgery

Back
ACUTE
|
COG criteria
|
anaplastic histology: stage III
1st line – 

surgery

Upfront nephrectomy using a transperitoneal approach is recommended for patients with unilateral renal masses who do not have a known Wilms' tumour predisposition.[3][40]

Surgeon should avoid tumour spillage or incomplete removal. Selective sampling of lymph nodes should also be performed.[72]

Palpation of renal vein and inferior vena cava identifies any tumour thrombi (usually free-floating) and may allow for careful removal of thrombus.[40]

Any suspicious metastatic lesion (hepatic or intra-abdominal) should be either biopsied or resected.[40]

Biopsy remains controversial, but an open biopsy with a minimum of 10-12 cores is required in the Children's Oncology Group (COG) protocol if a tumour is unresectable.[3][40]

Plus – postoperative chemotherapy

Back
ACUTE
|
COG criteria
|
anaplastic histology: stage III
Plus – 

postoperative chemotherapy

Treatment recommended for ALL patients in selected patient group

Treatment regimen depends on whether anaplasia is focal or diffuse.

Focal anaplasia: vincristine, dactinomycin, and doxorubicin (DD-4A regimen).[94]

Diffuse anaplasia: vincristine, doxorubicin, cyclophosphamide, carboplatin, and etoposide (revised UH-1 regimen).[83][95]

See local specialist protocol for dosing guidelines.

Primary options

DD-4A regimen

vincristine

and

dactinomycin

and

doxorubicin

OR

revised UH-1 regimen

vincristine

and

doxorubicin

and

cyclophosphamide

and

carboplatin

and

etoposide

Plus – radiotherapy

Back
ACUTE
|
COG criteria
|
anaplastic histology: stage III
Plus – 

radiotherapy

Treatment recommended for ALL patients in selected patient group

Patients with focal or diffuse anaplasia should receive abdomen/flank irradiation, with a boost to residual tumour.[83][94]

1st line – surgery

Back
ACUTE
|
COG criteria
|
anaplastic histology: stage IV
1st line – 

surgery

Upfront nephrectomy using a transperitoneal approach is recommended for patients with unilateral renal masses who do not have a known Wilms' tumour predisposition.[3][40]

Surgeon should avoid tumour spillage or incomplete removal. Selective sampling of lymph nodes should also be performed.[72]

Palpation of renal vein and inferior vena cava identifies any tumour thrombi (usually free-floating) and may allow for careful removal of thrombus.[40]

Any suspicious metastatic lesion (hepatic or intra-abdominal) should be either biopsied or resected.[40]

Biopsy remains controversial, but an open biopsy with a minimum of 10-12 cores is required in the Children's Oncology Group (COG) protocol if a tumour is unresectable.[3][40]

Plus – postoperative chemotherapy

Back
ACUTE
|
COG criteria
|
anaplastic histology: stage IV
Plus – 

postoperative chemotherapy

Treatment recommended for ALL patients in selected patient group

Patients with either focal or diffuse anaplasia receive revised UH-1 regimen, or UH-2 regimen (revised UH-1 with additional vincristine and irinotecan) in patients with poor/partial response to chemotherapy.[83][95]

See local specialist protocol for dosing guidelines.

Primary options

revised UH-1 regimen

vincristine

and

doxorubicin

and

cyclophosphamide

and

carboplatin

and

etoposide

OR

UH-2 regimen

vincristine

and

doxorubicin

and

cyclophosphamide

and

carboplatin

and

etoposide

and

irinotecan

Plus – radiotherapy

Back
ACUTE
|
COG criteria
|
anaplastic histology: stage IV
Plus – 

radiotherapy

Treatment recommended for ALL patients in selected patient group

Patients with either focal or diffuse anaplasia receive abdomen/flank irradiation, with a boost to residual tumour.[83][94] Patients with lung metastasis receive whole lung irradiation.

1st line – preoperative chemotherapy

Back
ACUTE
|
COG criteria
|
anaplastic histology: stage V
1st line – 

preoperative chemotherapy

In patients with bilateral Wilms' tumour or bilaterally predisposed unilateral Wilms' tumour, preoperative chemotherapy is followed by nephron-sparing surgery and modified postoperative chemotherapy based on histological response.[83][87][88]

The goal of preoperative chemotherapy is to shrink the tumour to allow maximum preservation of renal parenchyma.[88]

See local specialist protocol for regimen and dosing guidelines.

Plus – surgery

Back
ACUTE
|
COG criteria
|
anaplastic histology: stage V
Plus – 

surgery

Treatment recommended for ALL patients in selected patient group

Radical nephrectomies can potentially be avoided and nephron-sparing surgery (e.g., unilateral total nephrectomy with contralateral partial nephrectomy, bilateral partial nephrectomy, unilateral total nephrectomy, and unilateral partial nephrectomy) used to preserve renal parenchyma and function.[87]

Partial nephrectomy may not be sufficient for high-risk patients due to a high incidence of recurrence.[89][90][91]

Plus – postoperative chemotherapy

Back
ACUTE
|
COG criteria
|
anaplastic histology: stage V
Plus – 

postoperative chemotherapy

Treatment recommended for ALL patients in selected patient group

Postoperative chemotherapy is based on histological response.[83][87][88]

Each side should be staged individually and treated according to the guidelines above.

See local specialist protocol for regimen and dosing guidelines.

Consider – renal transplant

Back
ACUTE
|
COG criteria
|
anaplastic histology: stage V
Consider – 

renal transplant

Additional treatment recommended for SOME patients in selected patient group

Rarely, disease is extensive bilaterally resulting in renal failure and need for a renal transplant.[92]

SIOP criteria

1st line – preoperative chemotherapy

Back
ACUTE
|
SIOP criteria
|
stage I
1st line – 

preoperative chemotherapy

The UMBRELLA International Society of Paediatric Oncology (SIOP)-Renal Tumour Study Group (RTSG) 2016 protocol (known as the UMBRELLA protocol) recommends upfront chemotherapy for patients newly diagnosed with Wilms' tumour aged ≥6 months prior to any attempt at resection regardless of initial stage.[55][75]

In patients <6 months old, the UMBRELLA protocol accounts for the risk of misdiagnosis of Wilms' tumour by recommending direct surgery instead of preoperative chemotherapy.[41]

SIOP any risk: dactinomycin and vincristine (AV regimen) for 4 weeks.

See local specialist protocol for dosing guidelines.

Primary options

AV regimen

dactinomycin

and

vincristine

Plus – surgery

Back
ACUTE
|
SIOP criteria
|
stage I
Plus – 

surgery

Treatment recommended for ALL patients in selected patient group

After preoperative chemotherapy, radical tumour nephrectomy is the standard of care for all patients with Wilms' tumour.[75] However, the SIOP UMBRELLA protocol has specified certain conditions in which nephron-sparing surgery is acceptable for non-syndromic unilateral Wilms' tumours: small tumour volume (<300 mL) and the expectation of a substantial remnant kidney function in patients with tumours <300 mL who never had lymph node involvement.[75]

Surgeon should avoid tumour spillage or incomplete removal. Selective sampling of lymph nodes should also be performed.[72]

Palpation of renal vein and inferior vena cava identifies any tumour thrombi (usually free-floating) and may allow for careful removal of thrombus.[40]

Any suspicious metastatic lesion (hepatic or intra-abdominal) should be either biopsied or resected.[40]​

Consider – postoperative chemotherapy

Back
ACUTE
|
SIOP criteria
|
stage I
Consider – 

postoperative chemotherapy

Additional treatment recommended for SOME patients in selected patient group

Chemotherapy regimen depends on staging and histology of tumour.

SIOP histological classification divides patients with all stages (I to IV) into 3 groups: low-risk (completely necrotic Wilms' tumour), high-risk (blastemal type and diffuse anaplasia), and intermediate-risk tumours (all other types).[41][55]​​

SIOP low-risk: no postoperative chemotherapy.

SIOP intermediate-risk: dactinomycin and vincristine (AV regimen) for 4 weeks.

SIOP high-risk: dactinomycin, vincristine, and doxorubicin (AVD regimen) for 27 weeks.

See local specialist protocol for dosing guidelines.

Primary options

AV regimen

dactinomycin

and

vincristine

OR

AVD regimen

dactinomycin

and

vincristine

and

doxorubicin

1st line – preoperative chemotherapy

Back
ACUTE
|
SIOP criteria
|
stage II
1st line – 

preoperative chemotherapy

The UMBRELLA International Society of Paediatric Oncology (SIOP)-Renal Tumour Study Group (RTSG) 2016 protocol (known as the UMBRELLA protocol) recommends upfront chemotherapy for patients newly diagnosed with Wilms' tumour aged ≥6 months prior to any attempt at resection regardless of initial stage.[55][75]

In patients <6 months old, the UMBRELLA protocol accounts for the risk of misdiagnosis of Wilms' tumour by recommending direct surgery instead of preoperative chemotherapy.[41]

SIOP any-risk: dactinomycin and vincristine (AV regimen) for 4 weeks.

See local specialist protocol for dosing guidelines.

Primary options

AV regimen

dactinomycin

and

vincristine

Plus – surgery

Back
ACUTE
|
SIOP criteria
|
stage II
Plus – 

surgery

Treatment recommended for ALL patients in selected patient group

After preoperative chemotherapy, radical tumour nephrectomy is the standard of care for all patients with Wilms' tumour.[75] However, the SIOP UMBRELLA protocol has specified certain conditions in which nephron-sparing surgery is acceptable for non-syndromic unilateral Wilms' tumours: small tumour volume (<300 mL) and the expectation of a substantial remnant kidney function in patients with tumours <300 mL who never had lymph node involvement.[75]

Surgeon should avoid tumour spillage or incomplete removal. Selective sampling of lymph nodes should also be performed.[72]

Palpation of renal vein and inferior vena cava identifies any tumour thrombi (usually free-floating) and may allow for careful removal of thrombus.[40]

Any suspicious metastatic lesion (hepatic or intra-abdominal) should be either biopsied or resected.[40]

Plus – postoperative chemotherapy

Back
ACUTE
|
SIOP criteria
|
stage II
Plus – 

postoperative chemotherapy

Treatment recommended for ALL patients in selected patient group

Chemotherapy regimen depends on staging and histology of tumour.

SIOP histological classification divides patients with all stages (I to IV) into 3 groups: low-risk (completely necrotic Wilms' tumour), high-risk (blastemal type and diffuse anaplasia), and intermediate-risk tumours (all other types).[41][55]​​​

SIOP low-risk: dactinomycin and vincristine (AV regimen) for 27 weeks.

SIOP intermediate-risk (stromal or epithelial-type disease): AV regimen for 27 weeks.

SIOP intermediate-risk (non-stromal- or non-epithelial-type disease): dactinomycin, vincristine, and doxorubicin (AVD regimen) for 27 weeks.

SIOP high-risk: etoposide, carboplatin, cyclophosphamide, and doxorubicin (HR-1 regimen) for 34 weeks.

See local specialist protocol for dosing guidelines.

Primary options

AV regimen

dactinomycin

and

vincristine

OR

AVD regimen

dactinomycin

and

vincristine

and

doxorubicin

OR

HR-1 regimen

etoposide

and

carboplatin

and

cyclophosphamide

and

doxorubicin

Consider – radiotherapy

Back
ACUTE
|
SIOP criteria
|
stage II
Consider – 

radiotherapy

Additional treatment recommended for SOME patients in selected patient group

Flank irradiation is indicated in patients with high-risk diffuse anaplasia.[41]

1st line – preoperative chemotherapy

Back
ACUTE
|
SIOP criteria
|
stage III
1st line – 

preoperative chemotherapy

The UMBRELLA International Society of Paediatric Oncology (SIOP)-Renal Tumour Study Group (RTSG) 2016 protocol (known as the UMBRELLA protocol) recommends upfront chemotherapy for patients newly diagnosed with Wilms' tumour aged ≥6 months prior to any attempt at resection regardless of initial stage.[55][75]

In patients <6 months old, the UMBRELLA protocol accounts for the risk of misdiagnosis of Wilms' tumour by recommending direct surgery instead of preoperative chemotherapy.[41]

SIOP any risk: preoperative chemotherapy with dactinomycin and vincristine (AV regimen) for 4 weeks.

See local specialist protocol for dosing guidelines.

Primary options

AV regimen

dactinomycin

and

vincristine

Plus – surgery

Back
ACUTE
|
SIOP criteria
|
stage III
Plus – 

surgery

Treatment recommended for ALL patients in selected patient group

After preoperative chemotherapy, radical tumour nephrectomy is the standard of care for all patients with Wilms' tumour.[75] However, the SIOP UMBRELLA protocol has specified certain conditions in which nephron-sparing surgery is acceptable for non-syndromic unilateral Wilms' tumours: small tumour volume (<300 mL) and the expectation of a substantial remnant kidney function in patients with tumours <300 mL who never had lymph node involvement.[75]

Surgeon should avoid tumour spillage or incomplete removal. Selective sampling of lymph nodes should also be performed.[72]

Palpation of renal vein and inferior vena cava identifies any tumour thrombi (usually free-floating) and may allow for careful removal of thrombus.[40]

Any suspicious metastatic lesion (hepatic or intra-abdominal) should be either biopsied or resected.[40]

Plus – postoperative chemotherapy

Back
ACUTE
|
SIOP criteria
|
stage III
Plus – 

postoperative chemotherapy

Treatment recommended for ALL patients in selected patient group

Chemotherapy regimen depends on staging and histology of tumour.

SIOP histological classification divides patients with all stages (I to IV) into 3 groups: low-risk (completely necrotic Wilms' tumour), high-risk (blastemal type and diffuse anaplasia), and intermediate-risk tumours (all other types).[41][55]​​​

SIOP low-risk: dactinomycin and vincristine (AV regimen) for 27 weeks.

SIOP intermediate-risk (tumour volume after preoperative chemotherapy <500 mL of any subtype or tumour volume ≥500 mL of stromal or epithelial-type disease): AV regimen for 27 weeks.

SIOP intermediate-risk (tumour volume ≥500 mL of non-stromal- or non-epithelial-type disease): dactinomycin, vincristine, and doxorubicin (AVD regimen) for 27 weeks.

SIOP high-risk: etoposide, carboplatin, cyclophosphamide, and doxorubicin (HR-1 regimen) for 34 weeks.

See local specialist protocol for dosing guidelines.

Primary options

AV regimen

dactinomycin

and

vincristine

OR

AVD regimen

dactinomycin

and

vincristine

and

doxorubicin

OR

HR-1 regimen

etoposide

and

carboplatin

and

cyclophosphamide

and

doxorubicin

Plus – radiotherapy

Back
ACUTE
|
SIOP criteria
|
stage III
Plus – 

radiotherapy

Treatment recommended for ALL patients in selected patient group

SIOP low-risk: no irradiation.

SIOP intermediate-risk: flank irradiation is indicated.

SIOP high-risk: flank irradiation is indicated.

1st line – preoperative chemotherapy

Back
ACUTE
|
SIOP criteria
|
stage IV
1st line – 

preoperative chemotherapy

The UMBRELLA International Society of Paediatric Oncology (SIOP)-Renal Tumour Study Group (RTSG) 2016 protocol (known as the UMBRELLA protocol) recommends upfront chemotherapy for patients newly diagnosed with Wilms' tumour aged ≥6 months prior to any attempt at resection regardless of initial stage.[55][75]

In patients <6 months old, the UMBRELLA protocol accounts for the risk of misdiagnosis of Wilms' tumour by recommending direct surgery instead of preoperative chemotherapy.[41]

Chemotherapy regimen depends on staging and histology of tumour.

SIOP any risk: dactinomycin, vincristine, and doxorubicin (AVD regimen) for 6 weeks.

See local specialist protocol for dosing guidelines.

Primary options

AVD regimen

dactinomycin

and

vincristine

and

doxorubicin

Plus – surgery

Back
ACUTE
|
SIOP criteria
|
stage IV
Plus – 

surgery

Treatment recommended for ALL patients in selected patient group

After preoperative chemotherapy, radical tumour nephrectomy is the standard of care for all patients with Wilms' tumour.[75]

Surgeon should avoid tumour spillage or incomplete removal. Selective sampling of lymph nodes should also be performed.[72]

Palpation of renal vein and inferior vena cava identifies any tumour thrombi (usually free-floating) and may allow for careful removal of thrombus.[40]

Any suspicious metastatic lesion (hepatic or intra-abdominal) should be either biopsied or resected.[40]

Plus – postoperative chemotherapy

Back
ACUTE
|
SIOP criteria
|
stage IV
Plus – 

postoperative chemotherapy

Treatment recommended for ALL patients in selected patient group

Chemotherapy regimen depends on staging and histology of tumour.

SIOP histological classification divides patients with all stages (I to IV) into 3 groups: low-risk (completely necrotic Wilms' tumour), high-risk (blastemal type and diffuse anaplasia), and intermediate-risk tumours (all other types).[41][55]​​​

SIOP low- or intermediate-risk (complete or very good partial remission of metastatic lesions to preoperative chemotherapy; representative metastatic nodule resection feasible and had completely necrotic metastasis): dactinomycin, vincristine, and doxorubicin (AVD regimen) for 27 weeks.

SIOP low- or intermediate-risk (partial remission of metastatic lesions to preoperative chemotherapy and resection of metastatic nodules; representative nodule resection confirmed viable metastasis or metastatic nodule resection is not feasible): etoposide, carboplatin, cyclophosphamide, and doxorubicin (HR-1 regimen) for 34 weeks.

SIOP high-risk: discuss best current treatment approach with the principal investigator for stage IV disease.

See local specialist protocol for dosing guidelines.

Primary options

AVD regimen

dactinomycin

and

vincristine

and

doxorubicin

OR

HR-1 regimen

etoposide

and

carboplatin

and

cyclophosphamide

and

doxorubicin

Plus – radiotherapy

Back
ACUTE
|
SIOP criteria
|
stage IV
Plus – 

radiotherapy

Treatment recommended for ALL patients in selected patient group

Pulmonary radiotherapy is only administered for lung metastases lacking complete response by postoperative week 10 and in all cases with high-risk tumours, despite response to treatment.[3][75]

Consider irradiation to metastases in low-risk disease if resection of nodule resection is not feasible.

Irradiation to metastases in intermediate- and high-risk disease is indicated.

1st line – preoperative chemotherapy

Back
ACUTE
|
SIOP criteria
|
stage V
1st line – 

preoperative chemotherapy

In bilateral Wilms' tumour, preoperative chemotherapy with dactinomycin and vincristine (AV regimen) for a maximum of 12 weeks with evaluation of response at 6 weeks followed by surgery.

See local specialist protocol for regimen and dosing guidelines.

Primary options

AV regimen

dactinomycin

and

vincristine

Plus – surgery

Back
ACUTE
|
SIOP criteria
|
stage V
Plus – 

surgery

Treatment recommended for ALL patients in selected patient group

Nephron-sparing surgery is advocated for bilateral disease; this may include tumourectomy, wedge resection, polar resection, heminephrectomy, nephrectomy on one side, and partial resection, thus avoiding bilateral radical nephrectomies.[90]

The UMBRELLA protocol recommends discussion with the International Society of Paediatric Oncology-Renal Tumour Study Group (SIOP-RTSG) surgical panel to assess the feasibility of nephron-sparing surgery and minimise the risk of upstaging by incomplete resection of the tumour.[75]

Plus – postoperative chemotherapy

Back
ACUTE
|
SIOP criteria
|
stage V
Plus – 

postoperative chemotherapy

Treatment recommended for ALL patients in selected patient group

Each tumour is subclassified and staged separately to determine postoperative chemotherapy.

See local specialist protocol for regimen and dosing guidelines.

ONGOING

tumour recurrence

1st line – chemotherapy

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ONGOING
|
tumour recurrence
1st line – 

chemotherapy

Recurrent tumours are managed by use of chemotherapy agents that were not used for primary therapy.[75][96][97] The use of high-dose therapy with autologous stem cell transplantation or clinical trials utilising novel chemotherapy regimens may be considered.[75][98][99][100]

Consider – surgical resection

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ONGOING
|
tumour recurrence
Consider – 

surgical resection

Additional treatment recommended for SOME patients in selected patient group

Surgical resection of relapsed disease is considered when surgery seems possible or when it is useful to evaluate histological tumour response.[75]

Consider – radiotherapy

Back
ONGOING
|
tumour recurrence
Consider – 

radiotherapy

Additional treatment recommended for SOME patients in selected patient group

The use of radiotherapy to initially non-irradiated sites is accepted, but recommendations for the approach to previously irradiated sites is difficult, because of the many different situations encountered.[75]

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