Treatments for stage 4 Wilms tumour

The following are treatment options for stage 4 Wilms tumour. The healthcare team will suggest treatments based on your child’s needs and work with you to develop a treatment plan.

The healthcare team also considers the type of Wilms tumour when they plan treatments. Treatment for both favourable and anaplastic (unfavourable) stage 4 Wilms tumour usually involves surgery followed by radiation therapy and chemotherapy.

Surgery

Surgery is the main treatment for stage 4 Wilms tumour, followed by radiation therapy and chemotherapy. The most common surgery used is a radical nephrectomy (removal of the kidney) with lymph node removal.

Sometimes surgery cannot remove stage 4 kidney tumours completely because of their size or location. If the 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. 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 and radiation therapy are given.

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 the suspicious area to confirm that a spot in the lung seen on imaging is really a tumour. Surgery to remove a lung metastasis may also be done after chemotherapy and radiation therapy are given.

Radiation therapy

If the tumour is found in the lymph nodes or in the abdomen beyond the kidney, radiation therapy to the abdomen is given after surgery. Radiation may be given to one side of the abdomen 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.

Chemotherapy

If the tumour is removed with surgery, chemotherapy is given after surgery along with radiation therapy for stage 4 Wilms tumour.

If the tumour can’t be removed with surgery, chemotherapy may be given before surgery to shrink the tumour and then again after surgery. The type of chemotherapy most commonly used depends on the type of Wilms tumour.

Favourable histology tumours are treated with vincristine (Oncovin), doxorubicin (Adriamycin) and dactinomycin (Cosmegen, actinomycin-D). Chemotherapy is given for 25 weeks.

Favourable histology tumours with some chromosome changes may be treated with vincristine, doxorubicin, dactinomycin, cyclophosphamide (Procytox) and etoposide (Vepesid, VP-16) for 31 weeks.

Focal anaplastic tumours and diffuse anaplastic tumours are treated with vincristine, doxorubicin, etoposide, cyclophosphamide, carboplatin and irinotecan (Camptosar) for 36 weeks.

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

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.

Expert review and references

  • American Cancer Society. Treating Wilms Tumor. 2018.
  • Fernandez CV, Geller JI, Ehrlich PF, van den Heuvel-Eibrink, MM, 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 Version 1.2023 . 2023: https://www.nccn.org/guidelines/category_1.
  • PDQ Pediatric Treatment Editorial Board. Wilms Tumour and Other Childhood Kidney Tumors Treatment (PDQ®) – Health Professional Version. Bethesda, MD: National Cancer Institute; May 2023: https://www.cancer.gov/.
  • 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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