Treatments for stage 3 Wilms tumour

The following are treatment options for stage 3 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 3 Wilms tumour usually involves surgery followed by radiation therapy to the abdomen and chemotherapy.

Surgery

Surgery is the main treatment for stage 3 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 3 tumours completely because of their size or location. If the tumour can’t be removed safely because it is too large or has grown outside of the kidney into nearby tissue or blood vessels, a small tissue sample may be removed for biopsy and chemotherapy will be given. Within 6 weeks of starting chemotherapy, doctors will assess the tumour to find out whether a radical nephrectomy can be done. If not, further chemotherapy may be given to try to shrink the tumour some more. A reassessment should occur at 9 or 12 weeks from diagnosis to determine if the full tumour can be removed. Surgery or re-biopsy is encouraged no later than 12 weeks from diagnosis.

Radiation therapy

Once the tumour is removed by surgery, radiation therapy to the abdomen is given for stage 3 Wilms tumour. This may occur right at the beginning of treatment if the tumour is fully removed at diagnosis or after delayed surgery at week 6, 9 or 12. Radiation may be given to one side of the abdomen or to the entire abdomen. Chemotherapy is given with radiation therapy.

Chemotherapy

If the tumour is removed with surgery, chemotherapy is given with radiation therapy for stage 3 Wilms tumour.

If the tumour can’t be removed safely 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 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 are treated with vincristine, doxorubicin, dactinomycin, cyclophosphamide (Procytox) and etoposide (Vepesid, VP-16) for 31 weeks.

Focal anaplastic tumours are treated with vincristine, doxorubicin and dactinomycin for 25 weeks.

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

When cyclophosphamide is used, mesna (Uromitexan) may also 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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