Wilms tumour

"Wilms tumour | NHS inform Home Illnesses and conditions Symptoms and self-help Tests and treatments Healthy living Care, support and rights Scotlands Service Directory 0 Home Illnesses and conditions Cancer Cancer types in children Wilms tumour Wilms tumour Wilms tumour is a type of kidney cancer in children. It most often affects children under the age of 5. More children than ever are surviving childhood cancer. There are new and better drugs and treatments, and we can now also work to reduce the after-effects of having had cancer in the past. It is devastating to hear that your child has cancer, and at times it can feel overwhelming. There are many healthcare professionals and support organisations to help you through this difficult time. Understanding more about the cancer your child has and the treatments that may be used can often help parents to cope. Your childs specialist will give you more detailed information. If you have any questions its important to ask the specialist doctor or nurse who knows your childs individual situation. Wilms tumour is a type of kidney cancer that was named after Dr Max Wilms, who first described it. Its thought to come from very specialised cells in the embryo known as metanephric blastema. These cells are involved in the development of the childs kidneys while they are in the womb. The cells usually disappear at birth, but in many children with Wilms tumour, clusters of primitive kidneys cells, called nephrogenic rests can still be found. The kidneys are a pair of organs found at the back of the abdomen. They clean the blood by removing excess fluids and waste products, which are then converted into urine. Causes In most children, the causes of Wilms tumour are unknown. Very rarely, people who develop Wilms tumour have other specific conditions which are present at birth (congenital malformations). These include the lack of an iris in the eye (aniridia), abnormalities of the genitals, and a condition where one side of the body is slightly larger than the other (hemihypertrophy). In 1 in 100 people with Wilms tumour, another family member will also have Wilms tumour. Signs and symptoms The most common symptom is a swollen abdomen, which is usually painless. Sometimes a parent or carer may feel a lump in the abdomen which can be quite large. Occasionally, the tumour may bleed slightly and this can irritate the kidney and may be painful. There may be blood in your childs urine (pee), or their blood pressure may be raised. The child may also have a high temperature (fever), upset stomach, weight loss or a lack of appetite. How Wilms tumour is diagnosed A variety of tests and investigations may be needed to diagnose a Wilms tumour. An abdominal ultrasound scan is usually the first thing that is done. This will be followed by an MRI and/or CT scan of the abdomen and chest. These scans help doctors to identify exactly where the tumour is and whether it has spread beyond the kidney. This is known as staging. Urine and blood samples will also be taken to check your childs kidney function and general health. Most children will go on to have a biopsy , where a sample of tissue is taken from the tumour to confirm the diagnosis. Any tests and investigations that your child needs will be explained to you. Staging The stage of a cancer is a term used to describe its size and whether it has spread beyond its original site. Knowing the particular type and the stage of the cancer helps doctors decide on the most appropriate treatment. In the case of a Wilms tumour, the stage is finalised after surgery to remove the tumour. As most Wilms tumour patients receive chemotherapy before surgery, you may not know the exact stage of your childs tumour straight away. An exception is that babies under 6 months old usually have surgery straight away. Stage 1 The tumour is only affecting the kidney and has not begun to spread. It can be completely removed with surgery. Stage 2 The tumour has begun to spread beyond the kidney to nearby structures, but its still possible to remove it completely with surgery. Stage 3 The tumour has spread beyond the kidney; either because the tumour has burst before (or during) the operation, has spread to lymph glands (nodes), or has not been completely removed by surgery. Stage 4 The tumour has spread to other parts of the body such as the lungs or liver. Tumours in other parts of the body are known as metastases. Stage 5 There are tumours in both kidneys (bilateral Wilms tumour). If the tumour comes back after initial treatment, this is known as recurrent cancer or relapse. Treatment Treatment is planned by specialists in childrens tumours and cancers. This is usually based in a childrens oncology unit within a main hospital, but some treatment may be given closer to home. Treatment will depend on a number of factors including how the cells appear under the microscope (histology) and the stage of the tumour. Treatment may include chemotherapy, radiotherapy or surgery. Surgery All children with Wilms tumour will have surgery. Initially, this may only involve taking a small sample of cells from the tumour to confirm the diagnosis. This is called a biopsy and is usually done under a general anaesthetic using a needle inserted through the skin. Apart from very young children (under 6 months), most patients will receive chemotherapy before having a bigger operation to remove the entire tumour. The operation usually involves removing the whole of the affected kidney (nephrectomy). After examining the whole tumour under the microscope, Wilms tumours can be divided into a number of risk groups based on knowledge about how these different types of tumours are likely to behave. The treatment following surgery will depend on these risk groups. The risk groups are known as LOW, STANDARD (or INTERMEDIATE), and HIGH. The majority of tumours are in the standard risk group. So-called low risk tumours require less treatment than standard risk tumours. Two types of Wilms tumour  anaplastic and blastemal  are considered to be higher risk than other Wilms tumours and require more intensive (stronger) chemotherapy. Anaplastic Wilms tumour About 5% to 10% of Wilms tumours have an appearance called anaplasia, which means the cells look very disorganised under a microscope. Blastemal Wilms tumour This group of high-risk tumours cannot be identified by looking at the biopsy because they occur when a particular type of early kidney cell survives the pre-surgery chemotherapy. These cells are known as blastemal cells. Tumours, where most of these cells survive chemotherapy, are called blastemal tumours. Other kidney tumours Other, less common types of kidney tumours may occur in children. These are usually only recognised after surgery to obtain a tumour sample. Clear cell sarcoma and malignant rhabdoid tumour of the kidney are 2 types of cancerous tumour, with their own treatment recommendations. Congenital mesoblastic nephroma is a non-cancerous (benign) tumour that occurs in very young children. This type of tumour usually only needs surgery and no other treatment. Chemotherapy Chemotherapy is the use of anti-cancer (cytotoxic) drugs to destroy cancer cells. Its usually given as an injection or drip into a vein (intravenously). Chemotherapy given before surgery is called neoadjuvant chemotherapy. What doctors find out about the tumour after surgery for example, how the cells look under the microscope and how far the cancer has spread  helps them to decide whether additional chemotherapy should be given (adjuvant chemotherapy). This is to help reduce the risk of the cancer coming back (recurring). This chemotherapy may be given as inpatient or outpatient depending on tumour staging and risk group. Radiotherapy Radiotherapy treats cancer by using high-energy rays to destroy the cancer cells, while doing as little harm as possible to normal cells. Not all children with Wilms tumour need radiotherapy. For those that do, the area to be treated depends on the stage of the tumour at diagnosis. Some children receive radiotherapy to the area around the affected kidney or, less commonly, to the whole abdomen. If the tumour has spread to the lungs, then lung radiotherapy may be needed but this depends on how well the cancer responds to initial chemotherapy; its not always needed. Radiotherapy may occasionally be used to shrink tumours that are too large to remove surgically. This will, ideally, allow an operation to be done. Radiotherapy can also be used when tumours have spread elsewhere in the body. Treatment for bilateral Wilms tumour In about 1 in 20 cases, Wilms tumour affects both kidneys. Treatment usually involves surgery to both. The aim of the treatment is to remove as much of the cancer as possible, while leaving as much healthy kidney as possible. Chemotherapy is always given. Sometimes radiotherapy is needed as well. Side effects of treatment Treatment for Wilms tumour often causes side effects. Your childs doctor will discuss this with you before treatment starts. Many side effects are expected and managed effectively. Side effects can include feeling sick (nausea) and being sick (vomiting), hair loss, bruising and bleeding, tiredness, diarrhoea, and an increased risk of infection. Less common effects may include impact on the heart, kidneys, and liver. Late side effects A small number of children may develop late side effects, sometimes many years later. These include a possible reduction in bone growth, a change in the way the heart and lungs work, and a slight increase in their risk of developing another cancer in later life. Infertility is a possible late side effect, although this is rare. Your childs doctor or nurse will talk to you about any possible late side effects. These will depend on the exact treatments given. Clinical trials Many children have their treatment as part of a clinical research trial. Trials aim to improve our understanding of the best way to treat an illness. The trial may include comparing the standard treatment with a new or modified version. It may just involve collecting patient and tumour details including response to treatment to increase specialists knowledge. If appropriate, your childs medical team will talk to you about taking part in a clinical trial, and will answer any questions you have. Written information will be provided to help explain things. Taking part in a research trial is completely voluntary, and you will be given plenty of time to decide if its right for your child. Treatment guidelines Sometimes, clinical trials are not available for your childs tumour. This may be because a recent trial has just finished, or because the tumour is very rare. In these cases, you can expect your doctors and nurses to offer treatment which is agreed to be the most appropriate, using guidelines which have been prepared by experts across the country. The Childrens Cancer and Leukaemia Group (CCLG) is an important organisation which helps to produce these guidelines. Follow-up care Most children with Wilms tumour are cured. If the cancer comes back, its usually within the first 2 years. When one kidney is removed, the other will be able to work normally and can take over the work of the other kidney. Very few children have long-term kidney problems. Your child will have regular check-ups to look for any recurrence or problems following treatment. If you have specific concerns about your childs condition and treatment, its best to discuss them with your childs doctor, who knows the situation in detail. Your feelings As a parent, the fact that your child has cancer is one of the worst situations you can be faced with. You may have many emotions, such as fear, guilt, sadness, anger and uncertainty. These are all normal reactions and are part of the process that many parents go through at such a difficult time. Its not possible to address here all of the feelings you may have. However, the CCLG booklet Children & Young Peoples Cancer; A Parents Guide talks about the emotional impact of caring for a child with cancer and suggests sources of help and support. Your child may have a variety of powerful emotions throughout their experience with cancer. The Parents Guide discusses these further and talks about how you can support your child. Source: Children's Cancer and Leukaemia Group - Opens in new browser window Last updated: 14 November 2023 How can we improve this page? Help us improve NHS inform Thank You Your feedback has been received Dont include personal information e.g. name, location or any personal health conditions. Email Address e.g. you@example.com Message Maximum of 500 characters Send feedback Add this page to\n            Info For Me Search for cancer support services near you Enter a place or postcode NHS inform About NHS inform Editorial policy Contact us Webchat Give feedback about NHS inform Info for Me tool Terms and conditions Privacy and cookies policy Freedom of information (FOI) Accessibility Other languages and formats  2023 NHS 24  v1.1.1.17852"