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Wilms Tumor and Other Childhood Kidney Tumors Treatment (PDQ®)

Health Professional Version
Last Modified: 03/27/2014

Treatment of Wilms Tumor

Standard Treatment Options
Additional Treatment Considerations
        Stage I
        Stage III
        Stage IV
        Stage V and those predisposed to developing bilateral Wilms tumor
        Inoperable Wilms tumors
        Adults with Wilms tumor
Treatment Options Under Clinical Evaluation
        Stage I
        Stage II
        Stage III
        Stage IV
        Stage V and those predisposed to developing Wilms tumor
        Current Clinical Trials



Standard Treatment Options

Table 2 describes the standard chemotherapy regimens used to treat Wilms tumor.

Table 2. Standard Chemotherapy Regimens for Wilms Tumor
Regimen Name Regimen Description 
Regimen EE-4A [1]Vincristine, dactinomycin x 18 weeks postnephrectomy
Regimen DD-4A [1]Vincristine, dactinomycin, doxorubicin x 24 weeks postnephrectomy
Regimen I [2]Vincristine, doxorubicin, cyclophosphamide, etoposide x 24 weeks

Table 3 provides an overview of the standard treatment based on published results for all stages of Wilms tumor and survival information.

Table 3. Overview of Wilms Tumor Standard Treatment by Stage
Stage Histology 4 Year RFS or EFS 4 Year OS Treatment (see Table 2 for chemotherapy regimen descriptions) 
Stage I [1,2,4]FH <24 mo/tumor weight <550g85%98%Surgery only (should be done only within the context of a clinical trial)
FH >24 mo/tumor weight >550g94% RFS98%Nephrectomy + lymph node sampling followed by regimen EE-4A
DA68% EFS79%; (n = 10)Nephrectomy + lymph node sampling followed by regimen EE-4A and XRT
Stage II [1,2]FH86% RFS98%Nephrectomy + lymph node sampling followed by regimen EE-4A
FA80% EFS80%; (n = 5)Nephrectomy + lymph node sampling followed by abdominal XRT and regimen DD-4A
DA83% EFS82%Nephrectomy + lymph node sampling followed by abdominal XRT and regimen I
Stage III [1,2]FH87% RFS94%Nephrectomy + lymph node sampling followed by abdominal XRT and regimen DD-4A
FA88% RFS100%; (n = 8)Nephrectomy + lymph node sampling followed by abdominal XRT and regimen DD-4A
FA (preoperative treatment)71% RFS71%; (n = 7)Preoperative treatment with regimen DD-4A followed by nephrectomy + lymph node sampling and abdominal XRT
DA46% EFS53%; (n = 16)Preoperative treatment with regimen I followed by nephrectomy + lymph node sampling and abdominal XRT
DA65% EFS67%Immediate nephrectomy + lymph node sampling followed by abdominal XRT and regimen I
Stage IV [1,2]FH76% RFS86%Nephrectomy + lymph node sampling, followed by abdominal XRT,a bilateral pulmonary XRT,b and regimen DD-4A
FA61% EFS72%; (n = 11)Nephrectomy + lymph node sampling, followed by abdominal XRT,a bilateral pulmonary XRT,b and regimen DD-4A
DA33% EFS33%; (n = 15)Immediate nephrectomy + lymph node sampling followed by abdominal XRT,a whole-lung XRT,b and regimen I
DA (preoperative treatment)31% EFS44%; (n = 13)Preoperative treatment with regimen I followed by nephrectomy + lymph node sampling, followed by abdominal XRT,a and whole-lung XRTb
Stage V [1-3]Overall61% EFS80%; (n = 158)
FH65%87% (4-yr OS); 78% (10-yr OS)cBilateral renal biopsies and staging of each kidney followed by preoperative treatment with regimen EE-4A (if disease in both kidneys ≤ stage II) or regimen DD-4A (if disease in both kidneys > stage II), followed by second-look surgery and possibly more chemotherapy and/or XRTd
FA76%88%; (n = 9)Bilateral renal biopsies and staging of each kidney followed by preoperative treatment with regimen I, followed by second-look surgery and possibly more chemotherapy and/or XRTd
DA25%42%; (n = 20)Bilateral renal biopsies and staging of each kidney followed by preoperative treatment with regimen I, followed by second-look surgery and possibly more chemotherapy and/or XRT d

AH = anaplastic histology; DA = diffuse anaplastic; EFS = event-free survival; FA = focal anaplastic; FH = favorable histology; OS = overall survival; RFS = relapse-free survival; XRT = radiation therapy
aAbdominal XRT is planned according to local stage of renal tumor.
bPulmonary XRT is reserved for patients with chest x-ray evidence of pulmonary metastases.
c90% of the relapses occurred by 3.8 years from diagnosis and 90% of the deaths occurred within 5.7 years from diagnosis.[3]
dThis approach is changing as noted on the AREN0534 study.

Additional Treatment Considerations

Stage I

It may be possible to treat a subset of stage I Wilms tumor patients with surgery alone without chemotherapy. The Children’s Oncology Group (COG) addressed this question in the National Wilms Tumor Study-5 (NWTS-5 [COG-Q9401]) trial for children younger than 2 years at diagnosis with stage I favorable histology (FH) Wilms tumors that weigh less than 550 g. In the NWTS-5 study, the omission of adjuvant chemotherapy was tested for this group of patients.[4] Stringent stopping rules were designed to ensure closure of the study if the 2-year relapse-free survival rate was 90% or lower. The expectation was that approximately 50% of the surgery-only children would be salvaged after recurrence thus attaining the 95% predicted survival of children with very low-risk Wilms tumor treated with standard chemotherapy according to regimen EE-4A. This study was discontinued in 1998 when the predicted 2-year EFS fell below 90%.[5] Long-term follow-up of this study of the surgery-only cohort and the EE-4A group with a median follow-up of 8.2 years reported the estimated 5-year EFS for surgery only was 84% (95% confidence interval [CI], 73%–91%); for the EE-4A patients it was 97% (95% CI, 92%–99%, P = .002). One death was observed in each treatment group. The estimated 5-year overall survival (OS) was 98% (95% CI, 87%–99%) for surgery only and 99% (95% CI, 94%–99%) for EE-4A (P = .70).[4] COG study COG-AREN0532 is assessing this cohort again and is evaluating biological markers for this very low-risk group.[4,6]

Stage III

The outcome of patients with peritoneal implants treated with gross resection, three-drug chemotherapy, and total abdominal radiation (10.5 Gy) is similar to other stage III patients.[7][Level of evidence: 2A]

Stage IV

Stage IV disease is defined by the presence of hematogenous metastases to the lung, liver, bone, brain, or other sites, with the lung being the most common site. Previously, chest x-rays were used to detect pulmonary metastases. The introduction of computed tomography (CT) created controversy because many patients had lung nodules detected by chest CT scans that were not seen on chest x-rays. Management of newly diagnosed patients with FH Wilms tumor who have lung nodules detected only by CT scans (with negative chest x-ray) has elicited controversy as to whether they need to be treated with additional intensive treatment that is accompanied by acute and late toxicities. In a retrospective review of 186 patients from NWTS-4 and NWTS-5 (COG-Q9401) with CT-only detected lung nodules, patients who received doxorubicin in addition to vincristine and dactinomycin with or without lung radiation had a 5-year EFS of 80% versus 56% for patients receiving only two drugs (P = .004). There was no difference in the 5-year OS (87% vs. 86%).[8]

For patients with stage IV FH Wilms tumor, the role of pulmonary irradiation has been examined retrospectively (based on chest x-ray results) and is being examined prospectively (based on CT scan results) to identify clinical and radiologic features in patients that suggest that radiation can be omitted in certain subsets.

Investigators in the United Kingdom reviewed outcomes in children with stage IV Wilms tumor with pulmonary metastases at diagnosis and the factors that contributed to the decision to withhold pulmonary radiation. Patients who underwent pulmonary irradiation had a 9-year EFS of 79% versus 53% in patients who did not, although there was no difference in OS. Pulmonary radiation decreased the chance of lung relapse (8% vs 23%). No consistent features could be identified to aid in the selection of patients who could safely avoid pulmonary radiation.[9]

In a retrospective review of newly diagnosed patients with Wilms tumor and pulmonary metastases enrolled on the SIOP-93-01 and SIOP-WT-2001 studies, the 5-year OS was 83% and the 5-year EFS was 72% for all children (N = 207). Survival was poorer for high-risk primary tumor histology patients (5-year OS 44%, EFS 39%) than for low- and intermediate-risk patients (5-year OS 90%, EFS 77%). Complete response of patients with pulmonary metastases to 6 weeks of chemotherapy was associated with better outcome (5-year OS 91%, 5-year EFS 79%) compared with patients with stable or progressive disease (5-year OS and EFS 17%). The presence of viable tumor in the resected pulmonary metastases was associated with a poorer survival (5-year OS 55%, 5-year EFS 35%) compared with completely necrotic metastases (5-year OS 97%, 5-year EFS 85%). Of patients whose pulmonary lesions showed a complete remission to chemotherapy alone, approximately 20% relapsed.[10]

The presence of liver metastases at diagnosis is not an independent adverse prognostic factor in patients with stage IV Wilms tumor.[11]

Stage V and those predisposed to developing bilateral Wilms tumor

Management of a child with bilateral Wilms tumor is very challenging. The goals of therapy are to eradicate all tumor and to preserve as much normal renal tissue as possible, with the hope of decreasing the risk of chronic renal failure among these children.[12] Traditionally, patients have undergone bilateral renal biopsies with staging of each kidney. In NWTS-4, bilateral Wilms tumor patients had a lower EFS and OS compared with patients with localized Wilms tumor (including anaplastic histology), except for stage IV patients, in which OS was higher for patients with bilateral Wilms. The NWTS-4 study reported that the 8-year EFS for patients with bilateral Wilms tumor with favorable histology was 74% and the OS was 89%; for anaplastic histology, the EFS was 40% and OS was 45%.[13] The NWTS-5 (COG-Q9401) study reported the 4-year EFS for bilateral Wilms tumor patients was 61% and the OS was 81%; with anaplastic histology, the EFS was 44% and the OS was 55%.[2,3] Similar outcomes for patients with bilateral Wilms tumor have been reported in a single-institution experience in the Netherlands, with a 10-year OS of 78% (N = 41). In this study, there was significant morbidity in terms of renal failure (32%) and secondary tumors (20%).[14] The incidence of end-stage renal failure in the Dutch study may be a reflection of a longer follow-up period.

Treatment has changed from an initial surgical approach to an attempt to shrink the tumor and spare renal parenchyma with preoperative chemotherapy. The first COG trial to formally study bilateral Wilms tumors (COG-AREN0534) reflects the present recommendation to not perform an initial biopsy or laparotomy. Primary tumor excision should not be attempted, but patients should be given preoperative chemotherapy consisting of vincristine, dactinomycin, and doxorubicin. In a series of 49 patients with Wilms tumor who received preoperative therapy according to the SIOP-93-01 guidelines, the timing of surgery was determined when there was no longer imaging evidence of tumor regression. The mean treatment duration was 80 days prior to nephron-sparing surgery. The 5-year EFS rate was 83.4% and the OS rate was 89.5%. All but one of the patients had nephron-sparing surgery in at least one kidney. Despite the good survival, 14% of the patients developed end-stage renal disease.[15] In another series, nine out of ten patients with bilateral favorable-histology Wilms tumors underwent successful bilateral nephron-sparing procedures after receiving preoperative chemotherapy as detailed in a retrospective review from St. Jude Children’s Research Hospital.[16] One patient in the series developed renal failure after bilateral nephron-sparing surgery. Two patients with anaplastic histology died, although one patient died from complications of treatment rather than tumor. The OS for this group of patients was 83%. The authors recommend that bilateral nephron-sparing surgery should be considered for all patients who have bilateral Wilms tumor with favorable histology, even if preoperative imaging studies suggest that the lesions are unresectable.[16]

For patients who are treated with preoperative chemotherapy, it is essential to evaluate the tumor pathology after 4 to 8 weeks. The ideal time to do a biopsy or resection is not clear for patients who are not being treated on a protocol, since minimal shrinkage may reflect chemotherapy-induced differentiation or anaplastic histology. However, continuing therapy without evaluating tumor pathology in a patient with bilateral Wilms tumor may increase toxicity for the patient without providing additional benefit for tumor control. Anaplastic histology occurs in 10% of patients with bilateral Wilms tumor and responds poorly to chemotherapy. Once the diagnosis is made, a complete resection should be performed. Making the diagnosis is not straightforward, since in a series of 27 patients from NWTS-4, discordant pathology was seen in 20 cases, which highlights the need to obtain tissue from both kidneys. Seven children who were eventually found to have diffuse anaplastic tumors had core biopsies performed to establish the diagnosis but anaplasia was not found on the core biopsies. Anaplasia was identified in only three out of the nine patients when an open wedge biopsy was performed and in seven out of nine patients who had a partial or complete nephrectomy.[13]

Chemotherapy and/or radiation therapy following biopsy or second-look operation is dependent on the response to initial therapy, with more aggressive therapy required for patients with inadequate response to initial therapy observed at the second procedure or in the setting of anaplasia.[3,17-22]

Renal transplantation for children with Wilms tumor is usually delayed until 1 to 2 years have passed without evidence of malignancy.[23] Similarly, renal transplantation for children with Denys-Drash syndrome and Wilms tumor, all of whom require bilateral nephrectomy, is generally delayed 1 to 2 years after completion of treatment for the tumor.[23]

Inoperable Wilms tumors

In North America, standard therapy for Wilms tumor is primary nephrectomy and lymph node sampling followed by adjuvant chemotherapy. However, certain clinical presentations of Wilms tumor are referred to as inoperable Wilms tumor and include the following:

  • Metachronous bilateral Wilms tumor.
  • Wilms tumor in a solitary kidney.
  • Extension of tumor thrombus above the level of the hepatic veins.
  • Tumor involves contiguous structures whereby the only means of removing the kidney tumor requires removal of the other structures (e.g., spleen, pancreas, colon but excluding the adrenal gland).
  • Pulmonary compromise due to extensive pulmonary metastases.

Neoadjuvant chemotherapy consisting of vincristine, dactinomycin, and doxorubicin followed by resection and radiation therapy is the usual treatment for inoperable Wilms tumor. In the case of bilateral Wilms tumor or occurrence in a solitary kidney, the purpose of chemotherapy prior to surgery is to reduce the size of the tumor and allow preservation of maximal renal parenchyma at resection. In the case of extensive vena caval infiltration, initial chemotherapy also results in tumor shrinkage and minimizes the complications associated with subsequent resection and avoids the use of cardiopulmonary bypass.

Adults with Wilms tumor

In an analysis of Wilms tumor patients in the Surveillance, Epidemiology and End Results database, adults (n = 152) had a statistically worse OS (69% vs. 88%, P < .001) than pediatric patients (n = 2,190). Adults diagnosed with Wilms tumor were more likely than pediatric patients to be staged as having localized disease, to not receive any lymph node sampling, and to not receive any radiation treatment. The investigators recommended that all adult patients diagnosed with Wilms tumor should undergo lymph node sampling and that there should be close collaboration with pediatric surgeons and oncologists in treatment planning.[24]

Treatment Options Under Clinical Evaluation

Stage I

The following treatment options are currently under investigation in Children's Oncology Group (COG) clinical trials. Information about ongoing clinical trials is available from the NCI Web site.

Favorable Histology

  • COG-AREN0532 (Vincristine, Dactinomycin, and Doxorubicin With or Without Radiation Therapy or Observation Only in Treating Younger Patients Who Are Undergoing Surgery for Newly Diagnosed Stage I, Stage II, or Stage III Wilms Tumor): In this study, all tumors will be stratified based on central pathology review and molecular analysis (loss of heterozygosity at chromosomes 1p and 16q). Patients with loss of heterozygosity at 1p and 16q will be upstaged to receive treatment with regimen DD-4A (dactinomycin, doxorubicin, and vincristine for 24 weeks). Patients who are younger than 2 years and have Wilms tumors that weigh less than 550 g and who have a negative microscopic evaluation of lymph nodes are eligible for observation only. Other stage I patients will be treated with the standard therapy regimen EE-4A (dactinomycin and vincristine for 18 weeks) postnephrectomy.

Anaplastic (Focal or Diffuse) Histology

  • COG-AREN0321 (Combination Chemotherapy, Radiation Therapy, and/or Surgery in Treating Patients With High-Risk Kidney Tumors): In this study, patients with stage I will be treated with standard regimen DD-4A and radiation therapy.
Stage II

The following treatment options are currently under investigation in COG clinical trials. Information about ongoing clinical trials is available from the NCI Web site.

Favorable Histology

  • COG-AREN0532 (Vincristine, Dactinomycin, and Doxorubicin With or Without Radiation Therapy or Observation Only in Treating Younger Patients Who Are Undergoing Surgery for Newly Diagnosed Stage I, Stage II, or Stage III Wilms Tumor): In this study, all tumors will be stratified based on central pathology review and molecular analysis (loss of heterozygosity at chromosomes 1p and 16q). Patients with loss of heterozygosity at 1p and 16q will be upstaged to receive treatment with regimen DD-4A. Stage II patients without loss of heterozygosity will be treated with standard therapy regimen EE-4A postnephrectomy.

Focal Anaplastic

  • Patients with stage II will be treated with standard regimen DD-4A and radiation therapy.

Diffuse Anaplastic

  • COG-AREN0321 (Combination Chemotherapy, Radiation Therapy, and/or Surgery in Treating Patients With High-Risk Kidney Tumors): In this study, patients will be treated with the UH-1 regimen (cyclophosphamide, carboplatin, and etoposide alternating with vincristine, doxorubicin, and cyclophosphamide for 30 weeks) and radiation therapy.
Stage III

The following treatment options are currently under investigation in COG clinical trials. Information about ongoing clinical trials is available from the NCI Web site.

Favorable Histology

  • COG-AREN0532 (Vincristine, Dactinomycin, and Doxorubicin With or Without Radiation Therapy or Observation Only in Treating Younger Patients Who Are Undergoing Surgery for Newly Diagnosed Stage I, Stage II, or Stage III Wilms Tumor): In this study, patients will be treated with standard therapy regimen DD-4A and radiation therapy. Patients who have loss of heterozygosity at chromosomes 1p and 16q will be moved to clinical trial COG-AREN0533 (Combination Chemotherapy With or Without Radiation Therapy in Treating Young Patients With Newly Diagnosed Stage III or Stage IV Wilms Tumor) with regimen M (vincristine, dactinomycin, and doxorubicin alternating with cyclophosphamide and etoposide for a total of 24 weeks) and radiation therapy.

Focal Anaplastic

  • COG-AREN0321 (Combination Chemotherapy, Radiation Therapy, and/or Surgery in Treating Patients With High-Risk Kidney Tumors): In this trial, patients with stage III will be treated with standard regimen DD-4A and radiation therapy.

Diffuse Anaplastic

  • COG-AREN0321 (Combination Chemotherapy, Radiation Therapy, and/or Surgery in Treating Patients With High-Risk Kidney Tumors): In this trial, patients will be treated with the UH-1 regimen and radiation therapy.
Stage IV

The following treatment options are currently under investigation in COG clinical trials. Information about ongoing clinical trials is available from the NCI Web site.

Favorable Histology

  • COG-AREN0533 (Combination Chemotherapy With or Without Radiation Therapy in Treating Young Patients With Newly Diagnosed Stage III or Stage IV Wilms Tumor): In this trial, patients with pulmonary metastases only (detected by chest CT scans) will start treatment with standard chemotherapy regimen DD-4A and undergo abdominal irradiation if local stage III. Pulmonary metastases will be re-evaluated at 6 weeks with chest CT scan. Patients with complete resolution of pulmonary metastases will be considered rapid complete responders and will continue therapy with regimen DD-4A without any pulmonary radiation therapy. Patients who do not have a complete response (slow incomplete responders) will be switched to regimen M (for a total of 24 weeks) and undergo radiation therapy to their lungs. It is recommended that biopsies of residual pulmonary lesions be performed before radiation therapy is delivered.

    Patients with loss of heterozygosity at chromosomes 1p and 16q will be treated with regimen M with radiation therapy to all sites of disease. Patients with metastases outside or in addition to lung metastases will be treated with regimen M and radiation therapy.

Focal Anaplastic

  • COG-AREN0321 (Combination Chemotherapy, Radiation Therapy, and/or Surgery in Treating Patients With High-Risk Kidney Tumors): In this trial, patients will be treated with the UH-1 regimen and radiation therapy.

Diffuse Anaplastic (No Measurable Disease)

  • COG-AREN0321 (Combination Chemotherapy, Radiation Therapy, and/or Surgery in Treating Patients With High-Risk Kidney Tumors): In this trial, patients will be treated with the UH-1 regimen and radiation therapy.

Diffuse Anaplastic (Measurable Disease)

  • COG-AREN0321 (Combination Chemotherapy, Radiation Therapy, and/or Surgery in Treating Patients With High-Risk Kidney Tumors): In this trial, patients will be treated with window therapy consisting of vincristine and irinotecan for 12 weeks. If they respond to the window therapy, they will receive therapy consisting of UH-2 (cyclophosphamide, carboplatin, and etoposide; vincristine, doxorubicin, and cyclophosphamide; vincristine, irinotecan, and radiation therapy) for 30 weeks. Patients not responding to the window therapy would then be treated on UH-1 and radiation therapy.
Stage V and those predisposed to developing Wilms tumor

The following treatment option is currently under investigation in COG clinical trials. Information about ongoing clinical trials is available from the NCI Web site. Patients with multicentric tumors, patients with high-risk bilateral tumors, and patients with diffuse hyperplastic nephrogenic rests are treated on the following protocol:

COG-AREN0534 (Combination Chemotherapy and Surgery in Treating Young Patients With Wilms Tumor): Children with bilateral tumors are eligible for COG-AREN0534, which is the first protocol to prospectively study bilateral tumors. The goals of therapy are to eradicate all tumor and to preserve as much normal renal tissue as possible with the hope of decreasing the risk of chronic renal failure among these children.[25,26] When children are identified with bilateral tumors by CT or magnetic resonance imaging, central radiologic review will be performed to exclude tumor extension, invasion, rupture, metastases, or thrombus. Central review will also assess characteristics of nephrogenic rests versus tumor and differentiate active from sclerotic rests or tumors. Biopsy will not be mandated. Upfront intensification with three drugs (vincristine, doxorubicin and dactinomycin), will be used in large part to move patients earlier to definitive surgery. Repeat imaging will be mandated at 6 weeks. Based on response to treatment surgery, biopsy or continued chemotherapy will be performed. If biopsy or surgery is performed, chemotherapy or radiation therapy will be given based on histology. Repeat imaging will be performed at 12 weeks. If there is a complete response, definitive surgery or continued therapy will be performed. This approach will identify patients with anaplasia, rhabdomyomatous differentiation, complete necrosis, or stromal differentiation, select them for early surgery, and define the intensity of chemotherapy to be administered.[16,18,27] Chemotherapy and/or radiation therapy following the second-look operation is dependent on the response to initial therapy, with more aggressive therapy required for patients with inadequate response to initial therapy observed at the second procedure.[17-22]

Current Clinical Trials

Check for U.S. clinical trials from NCI's list of cancer clinical trials that are now accepting patients with stage I Wilms tumor, stage II Wilms tumor, stage III Wilms tumor, stage IV Wilms tumor, stage V Wilms tumor and recurrent Wilms tumor and other childhood kidney tumors. The list of clinical trials can be further narrowed by location, drug, intervention, and other criteria.

General information about clinical trials is also available from the NCI Web site.

References
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