Treatments for stage 4 Wilms tumour

Last medical review:

The following are treatment options for stage 4 Wilms tumour. The healthcare team will suggest treatments based on the type of Wilms tumour (whether the tumour has a favourable or anaplastic histology) and your childʼs needs. They will work with you to develop a treatment plan.

Surgery

Surgery is the main treatment for stage 4 Wilms tumour. The most common surgery used is a radical nephrectomy. A radical nephrectomy removes the whole kidney along with nearby structures. Lymph nodes near the kidney are also removed. Chemotherapy and radiation therapy are usually given after surgery.

Sometimes stage 4 Wilms tumours canʼt be completely removed with surgery because of their size or location. If a tumour can’t be removed safely because it is too large or has grown into nearby tissues or blood vessels, a small tissue sample may be removed for biopsy to check the histology of the tumour. Chemotherapy will be given to try to shrink the tumour. If chemotherapy shrinks the tumour enough that it can be removed, surgery is done and then more chemotherapy is given. Radiation therapy may also be used.

For Wilms tumour that has spread to the liver, surgery may be used to remove any liver tumours that remain after chemotherapy and radiation therapy.

Surgery may also be used to remove cancer that has spread (metastasized) to the lungs. This surgery may be done as part of diagnosis. The surgeon will remove tissue to confirm that a suspicious spot in the lung seen on imaging is cancer. Chemotherapy and radiation therapy may be given before surgery, if surgery is used to remove cancer that has spread to the lungs.

Find out more about surgery for Wilms tumour.

Radiation therapy

Radiation therapy uses high-energy rays or particles to destroy cancer cells.

If Wilms tumour has spread outside of the kidney to the lymph nodes or the abdomen, radiation therapy is given after surgery. Radiation therapy may be given to the area where the kidney was removed or to the entire abdomen. Chemotherapy is given with radiation therapy.

Radiation therapy may also be used to treat Wilms tumour that has spread to the lungs or to other locations in the body.

Find out more about radiation therapy for Wilms tumour.

Chemotherapy

Chemotherapy uses drugs to destroy cancer cells.

After a stage 4 Wilms tumour has been removed with surgery, chemotherapy is given with radiation therapy.

If the tumour can’t be removed with surgery, chemotherapy may be given before surgery to shrink the tumour, and then given again after surgery.

The chemotherapy drugs used depend on the type of tumour. It also depends on how well cancer in the lungs responded to initial treatment with chemotherapy.

For stage 4 Wilms tumour that has spread to the lungs, imaging tests will be done after 6 weeks of chemotherapy with vincristine, dactinomycin and doxorubicin.

If there is no visible cancer in the lungs after the initial treatment, this is referred to as a rapid, or complete, early response to treatment.

If there is cancer still visible in the lungs, this is referred to as a slow, early response to treatment.

Favourable histology tumours with a rapid, early response to treatment are treated with vincristine, doxorubicin and dactinomycin. Chemotherapy is given for 27 weeks (9 cycles) after surgery.

Favourable histology tumours with chromosome changes and a slow, early response to treatment or metastases in locations other than the lungs are treated with vincristine, doxorubicin, dactinomycin, cyclophosphamide and etoposide for about 33 weeks after surgery.

Focal anaplastic tumours are treated with vincristine, doxorubicin and dactinomycin for 27 weeks (9 cycles) after surgery.

Diffuse anaplastic tumours are treated with vincristine, doxorubicin, etoposide, cyclophosphamide and carboplatin for about 42 weeks (14 cycles) after surgery.

When cyclophosphamide is used, the drug mesna may be given. Mesna helps protect the bladder from irritation from cyclophosphamide.

Find out more about chemotherapy for Wilms tumour.

Clinical trials

Children with cancer may be treated in a clinical trial. Clinical trials look at new ways to prevent, find and treat cancer. Find out more about clinical trials.

Advanced cancer

Treatment is successful for many children with cancer, but in some cases it isnʼt. When cancer is advanced, the focus of treatment can change from cure to quality of life. There is help and support available. Find out more about when a cure is not possible.

Expert review and references

  • Meghan Pike, MD, FRCPC
  • American Cancer Society. Wilms Tumor. 2025: https://www.cancer.org/.
  • Dix DB, Seibel NL, Chi YY, et al. Treatment of stage IV favorable histology wilms tumor with lung metastases: A report from the Children's Oncology Group AREN0533 study. Journal of Clinical Oncology. 2018: 36(16):1564–1570.
  • Fernandez CV, Geller JI, Ehrlich PF, et al. Renal tumors. Blaney SM, Adamson PC, Helman LJ (eds.). Pizzo and Pollack's Pediatric Oncology. 8th ed. Wolters Kluwer; 2021: Kindle version, [chapter 24] https://read.amazon.ca/?asin=B08DVWZNVP&language=en-CA.
  • National Comprehensive Cancer Network. NCCN Clinical Practice Guidelines in Oncology: Wilms Tumor (Nephroblastoma) Version 2.2025. 2025: https://www.nccn.org/home.
  • Nelson MV, van den Heuvel-Eibrink MM, Graf N, Dome JS. New approaches to risk stratification for Wilms tumor. Current Opinions in Pediatrics. 2020: 33(1):40–48.
  • Ortiz MV, Koenig C, Armstrong AE, et al. Advances in the clinical management of high-risk Wilms tumors. Pediatric Blood and Cancer. 2023: 70(Suppl 2):e30342.
  • Paulino AC. Medscape Reference: Wilms Tumor. 2024: https://www.medscape.com/.
  • PDQ® Pediatric Treatment Editorial Board. Wilms Tumor and Other Childhood Kidney Tumors Treatment (PDQ®) – Health Professional Version. Bethesda, MD: National Cancer Institute; 2025: https://www.cancer.gov/.
  • PDQ® Pediatric Treatment Editorial Board. Wilms Tumor (PDQ®) – Patient Version. Bethesda, MD: National Cancer Institute; 2025: https://www.cancer.gov/.
  • Theilen TM, Braun Y, Bochennek K, Rolle U, Fiegel HC, Friedmacher F. Multidisciplinary treatment strategies for Wilms tumor: Recent advances, technical innovations and future directions. Frontiers in Pediatrics. 2022: 10:852185.
  • Wang J, Li M, Tang D, Gu W, Mao J, Shu Q. Current treatment for Wilms tumor: COG and SIOP standards. World Journal of Pediatric Surgery. 2019: 2:e000038.

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