Diagnosis & Treatment
Diagnosing Wilms is fairly straightforward and early indicators can be picked up from simple blood, urine and tissue samples.
Tackling Wilms, depending on the stage of diagnosis, can either be very straightforward or agonizingly complex.
The disease has a good survival rate if detected early but if diagnosed late the cancer can be fatal. Sadly, many like William go undiagnosed for years and face an uphill struggle to overcome the disease. They can require extensive, life-threatening surgical operations and many months or years of painful and debilitating treatments such as: chemotherapy, radiation and stem-cell harvesting and transplants.
The most common symptom of a Wilms’ tumor is a swollen stomach. This can occur quite suddenly and is usually painless to your child. Most Wilms' tumors are quite large when they are found. They are very often much bigger than the kidney itself. Other less common symptoms include:
Bleeding inside the tumor causing pain
Blood in the urine – found in between 15 and 20 out of every 100 children (15 to 20%) with a Wilms' tumor
Raised blood pressure
High temperature (fever) and also regular low-grade fever (37.0-37.5)
Loss of appetite
Feeling or being sick
Shortness of breath and cough (only if the cancer has spread to the lungs)
If possible, the tumor will be removed by surgery after a course of chemotherapy. This may involve removing part of or the entire kidney. The is called a 'nephrectomy'.
Your child’s surgeon will aim to keep as much of the healthy kidney as possible.
If Wilms’ tumors are in both kidneys then each kidney will be operated on separately.
Recovery after surgery should take between three and six weeks. Your doctor or surgeon will discuss the success of the surgery and any follow-up treatments you may need. Your doctor will monitor the function of the remaining kidney.
Wilms (or ‘nephroblastoma’): Surgical 'nephrectomy'
Chemotherapy & Radiation Therapy
Chemotherapy involves using anti-cancer drugs to target the cancer cells. Very often chemotherapy is given before surgery to shrink the tumor and then after surgery to stop the cancer from returning.
Radiotherapy may also be given, depending on the stage of the tumor. This involves targeting the tumour with high-energy rays.
If your child is undergoing chemotherapy, he or she may experience side-effects such as tiredness, sickness, diarrhoea and temporary hair loss. Your child may also be at greater risk of infections. Radiotherapy can also make your child feel tired and sick. He or she might experience skin irritation similar to sun burn and hair loss in the area that is being treated. Talk to your specialist or nurses about how you can manage these side-effects.
Wilms (or ‘nephroblastoma’): Chemotherapy treatment
After your treatment is complete your child will still need to be seen regularly at an outpatient clinic. At this clinic, your child’s specialist will be looking for any signs that the cancer has returned (this is known as a relapse). They will also check that your child’s major organs, such as the heart and lungs, are still functioning correctly and haven’t been affected by the cancer treatment.
Long-term follow up
Once your child’s risk of relapse has reduced, the focus of on-going follow up changes to looking out for potential long term side effects of treatment. The risk of long term side effects depends on the type of cancer your child had and the treatment received. A personalized surveillance plan is usually created that outlines the specific long term follow up and on-going investigations that your child will require. Many people are at low risk of future health problems but some will have significant on-going health needs. Talk to your child’s specialist about the potential long-term side-effects of their treatment.
When one kidney has been removed, the other will still function normally and will take over the work of the kidney that has been removed. Because Wilms’ tumours usually occur in one kidney only, very few children go on to have long-term kidney problems. Other late effects can include:
Heart or lung problems after receiving certain chemotherapy drugs or radiation therapy to these parts of the body
Slowed or decreased growth and development
Changes in sexual development and ability to have children (especially in girls)
Development of second cancers later in life (rare)
Diagnosis & Detection
The diagnosis of Wilms’ tumors usually involves a biopsy. A biopsy is where a sample of the tissue will be taken and examined under a microscope.
Urine samples will be taken to check how your child’s kidneys are functioning and ultrasound or CT scans will be used to see whether the cancer has spread.
The treatment of a Wilms’ tumor will depend on its size, whether or not it has spread to other parts of the body and the type of cells involved.
In the USA children follow the NWTSG/COG protocol and are treated with surgery first and then chemotherapy.
Children in Europe are usually treated with chemotherapy to shrink the tumor and then surgery.
Research has shown negligible differences between these two approaches.