The following are treatment options for recurrent Wilms tumour. The healthcare team will suggest treatments based on your child’s needs and work with you to develop a treatment plan. When planning treatment, the healthcare team will consider:

• treatments the child has already received
• the type of Wilms tumour (if it is favourable or unfavourable histology)
• how much time has passed since treatment
• where the cancer recurs (metastatic Wilms tumour can occur in the lungs, liver or opposite kidney and rarely other parts of the body)

Treatment may involve surgery, chemotherapy, radiation therapy or a stem cell transplant.

Treatment by level of risk

Three levels of risk have been identified for recurrent Wilms tumour based on certain prognostic factors and outcome after recurrence.

Standard risk

Tumours considered standard risk have a favourable histology. The primary tumour was treated with vincristine (Oncovin) and dactinomycin (Cosmegen, actinomycin-D).

Children with standard-risk tumours are usually treated with surgery, if it is possible to remove the recurrence and to confirm that the tumour is the same histology. Radiation therapy and chemotherapy are given after surgery or if surgery is not possible. The drugs used are vincristine (Oncovin), doxorubicin (Adriamycin) and cyclophosphamide (Cytoxan, Procytox), alternating with etoposide (Vepesid) and cyclophosphamide.

High risk

Tumours considered high risk also have a favourable histology. The primary tumour was treated with 3 or more chemotherapy drugs, with or without radiation therapy. Children with a short time to recurrence are also considered high risk.

Children with high-risk tumours are usually treated with surgery, if it is possible to remove the recurrence. Radiation therapy and chemotherapy are given after surgery or if surgery is not possible. There is no standard regimen that is used for high risk Wilms tumours. Some of chemotherapy drugs used are cyclophosphamide and etoposide, alternating with carboplatin (Paraplatin, Paraplatin AQ) and etoposide. Stem cell translplant may be used following chemotherapy.

Very high risk

Tumours considered very high risk have an unfavourable (anaplastic) histology. Children with a particular type of histology called blastemal predominant or a very short time to recurrence are also considered very high risk.

There is no standard regimen for very high risk Wilms tumours. Children with very high-risk tumours may be treated with more aggressive chemotherapy, such as ifosfamide (Ifex), carboplatin and etoposide. They may also be given high-dose chemotherapy followed by a stem cell transplant. They may also be offered additional treatments through a clinical trial setting.

Surgery

Surgery may be done to confirm the diagnosis and to remove a local recurrence.

Radiation therapy

Radiation therapy may be used to treat a recurrence in areas that were not already treated with radiation therapy.

Chemotherapy

Chemotherapy combinations that may be used for recurrent Wilms tumour depend on which chemotherapy drugs the child has already received and if the recurrent tumour has favourable or unfavourable (anaplastic) histology.

Stem cell transplant

Stem cell transplant may be a treatment option for children with a poor prognosis. Although this treatment approach continues to be used in children with high-risk recurrent Wilms tumour, there is controversy as to whether or not high-dose chemotherapy followed by stem cell transplant improves outcomes for children with Wilms compared to treatment with intensive chemotherapy alone.

Clinical trials

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.