How is it treated and managed?
Overview
Treatment of children with Wilms tumour will be at regional paediatric cancer centres that may be some distance from the child’s home. Type and duration of treatment will be based on two factors:
- stage of tumour,
- histology – low, intermediate or high risk groups.
Most children over the age of 6 months will begin their treatment with 4 weeks of pre-operative chemotherapy followed by surgery. Most children will have some post operative chemotherapy treatment as well. Wilms tumour is rare in children under 6 months - they usually have surgery as the first stage of their treatment.
The likely treatments are listed using staging information but this will not be enough information to work out the duration of treatment in some cases. In these cases, the total duration of treatment needed will not become clear until after the child has had surgery to remove their tumour. Care needs relate directly to type and duration of treatment and recovery time.
Stage 1 low and intermediate risk groups
Children will undergo 4 weeks chemotherapy before surgery and 0 or 4 weeks chemotherapy after surgery. Duration of stay in hospital after surgery is one week. Typical chemotherapy agents used include:
- Vincristine.
- Actinomycin D.
Stage 1 with high risk disease, stages 2 and 3
These children undergo preoperative chemotherapy for 4 weeks followed by surgery. Following surgery they are likely to remain in hospital for a week. Adjuvant chemotherapy begins after surgery and lasts for varying periods up to 34 weeks depending on the risk group of the tumour. The following drugs are likely to be used:
- Vincristine.
- Actinomycin D.
- Doxorubicin.
Stage 4
This group begins treatment with 6 weeks of chemotherapy prior to surgery. They will then undergo radical surgery to remove the kidney and surrounding tissue, recovering in hospital for a week or more afterwards. Further treatment may include radiotherapy to the kidney area or abdomen and/or radiotherapy to the lungs. Chemotherapy follows usually for up to 34 weeks depending on the features of the tumour and includes:
- Vincristine.
- Actinomycin D.
- Doxorubicin.
And it may also include:
- Carboplatin.
- Etoposide.
- Cyclophosphamide.
Children in this group may undergo a treatment protocol that lasts up to a year.
Stage 5
This group of children have a Wilms tumour in each kidney. Radical surgery to remove both kidneys would put the child on kidney dialysis. Treatment plans aim to avoid this by shrinking tumours with chemotherapy prior to surgery. In most cases one tumour is smaller than the other and part of one or both kidneys can be preserved. This group of children begin treatment with biopsy of their tumours followed by 6 weeks of chemotherapy and rescanning to plan surgery. Using this approach, some of one or both kidneys can be preserved. Children will have further chemotherapy or radiotherapy depending on the size and extent of the tumour in each kidney. Adjuvant chemotherapy may extend for up to 34 weeks.
Recurrence of Wilms Tumour
Wilms tumour may recur in the abdomen or other areas of the body such as the lungs. Survival following treatment for recurrence is variable, being good in some groups and poor in others. Treatment is likely to be tailored to the site of the recurrence and take account of treatment already given for the primary tumour. For example, if no radiotherapy has been given, radiotherapy may be used. Most children will undergo a combination of radiotherapy, surgery where possible and chemotherapy. Some children may undergo high dose chemotherapy and stem cell transplant.
