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The following are treatment options for stage IV 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 histology and unfavourable (anaplastic) histology usually involves surgery, followed by radiation therapy and chemotherapy.
If the tumour can’t be removed safely because it is too large or has grown into nearby vessels or structures, a small tissue sample may be removed for biopsy. Chemotherapy, with or without radiation therapy, 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. (Radiation therapy is not given again if it was given before surgery.)
Surgery is the primary treatment for both types of stage IV Wilms tumour. The type of surgery is radical nephrectomy and removal of nearby lymph nodes. If the tumour can’t be removed safely, a small tissue sample will be removed for biopsy.
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 be also be used to remove cancer that has spread, or 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 be done after chemotherapy and radiation therapy are given.
Radiation therapy is given to the abdomen after surgery for stage IV Wilms tumour. Radiation may be given to one side, or flank, of the abdomen or to the entire abdomen. It is used to kill any cancer cells that remain after surgery. Chemotherapy is given along with radiation therapy.
Radiation therapy may be given before surgery if the tumour is too large or has grown into nearby vessels or structures so that it can’t be removed with surgery. Radiation therapy, along with chemotherapy, is used to try to shrink the tumour and make surgery possible.
Radiation therapy is also used to treat Wilms tumour that has spread to the lungs.
Chemotherapy is given after surgery, along with radiation therapy, for stage IV Wilms tumour. For Wilms tumour that 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 chromosome changes are treated with vincristine, doxorubicin and dactinomycin for 25 weeks. Some children receiving treatment in a clinical trial may also receive cyclophosphamide (Cytoxan, Procytox) and etoposide (Vepesid) in addition to these drugs.
If doctors can still see a tumour in the lungs after 6 weeks of chemotherapy with these drugs, then vincristine, doxorubicin and dactinomycin will be given for another 6 weeks. At the end of this treatment, vincristine, dactinomycin, doxorubicin, cyclophosphamide (Cytoxan, Procytox) and etoposide (Vepesid) are given for a total of 31 weeks.
Focal anaplastic tumours are treated with either of the following for 25 weeks:
Diffuse anaplastic tumours are treated with vincristine, doxorubicin, etoposide, cyclophosphamide and carboplatin for 25 weeks. Some children receiving treatment in a clinical trial may receive irinotecan and vincristine followed by vincristine, doxorubicin, etoposide, cyclophosphamide and carboplatin.
When cyclophosphamide is used, mesna (Uromitexan) is also given. Mesna helps protect the bladder from irritation from cyclophosphamide.
Many children with Wilms tumour will be treated in a clinical trial that is tailored to the stage and type of tumour. The clinical trial protocol outlines the chemotherapy drugs and dosages that are used. Find out more about clinical trials.