TREATMENT

WILMS TUMOR

Staging and treatment

Staging is determined by combination of imaging studies and pathology findings if the tumor is
operable (adapted from www.cancer.gov). Treatment strategy is determined by the stage:

Stage I (43% of patients)

For stage I Wilms' tumor, 1 or more of the following criteria must be met:

Tumor is limited to the kidney and is completely excised.

The surface of the renal capsule is intact.

The tumor is not ruptured or biopsied (open or needle) prior to removal.

No involvement of renal sinus vessels.

No residual tumor apparent beyond the margins of excision.

Treatment: Nephrectomy +/- 18 weeks of chemotherapy depending on age of patient and weight
of tumor. EG: less than 2 years old and less than 550g only requires Nephrectomy with
observation
Outcome: 98% 4-year survival; 85% 4-year survival if anaplastic

[ Stage II (23% of patients)

For Stage II Wilms' tumor, 1 or more of the following criteria must be met:

Tumor extends beyond the kidney but is completely excised.

No residual tumor apparent at or beyond the margins of excision.

Any of the following conditions may also exist:

o Tumor involvement of the blood vessels of the renal sinus and/or outside the renal
parenchyma.
o The tumor has been biopsied prior to removal or there is local spillage of tumor
during surgery, confined to the flank.

Treatment: Nephrectomy + abdominal radiation + 24 weeks of chemotherapy

Outcome: 96% 4-year survival; 70% 4-year survival if anaplastic

Stage III (23% of patients)

For Stage III Wilms' tumor, 1 or more of the following criteria must be met:

Unresectable primary tumor.

Lymph node metastasis.

Positive surgical margins.

Tumor spillage involving peritoneal surfaces either before or during surgery, or transected
tumor thrombus.

Treatment: Abdominal radiation + 24 weeks of chemotherapy + nephrectomy after tumor

shrinkage
Outcome: 95% 4-year survival; 56% 4-year survival if anaplastic

Stage IV (10% of patients)

Stage IV Wilms' tumor is defined as the presence of hematogenous metastases (lung, liver, bone,
or brain), or lymph node metastases outside the abdomenopelvic region.
Treatment: Nephrectomy + abdominal radiation + 24 weeks of chemotherapy + radiation of
metastatic site as appropriate
Outcome: 90% 4-year survival; 17% 4-year survival if anaplastic

Stage V (5% of patients)

Stage V Wilms tumor is defined as bilateral renal involvement at the time of initial diagnosis.
Note: For patients with bilateral involvement, an attempt should be made to stage each side
according to the above criteria (stage I to III) on the basis of extent of disease prior to biopsy.
The 4-year survival was 94% for those patients whose most advanced lesion was stage I or stage
II; 76% for those whose most advanced lesion was stage III.
Treatment: Individualized therapy based on tumor burden

Stage I-IV Anaplasia

Children with stage I anaplastic tumors have an excellent prognosis (80-90% five-year survival).
They can be managed with the same regimen given to stage I favorable histology patients.
Children with stage II through stage IV diffuse anaplasia, however, represent a higher-risk group.
These tumors are more resistant to the chemotherapy traditionally used in children with Wilms
tumor (favorable histology), and require more aggressive regimens.