The most common form of kidney cancer is renal cell carcinoma or RCC for short. About 90 per cent of kidney cancers are RCCs. There are several different sub-types of RCC, which are named according to the type of cell that is affected, or the appearance of the cancer cells under the microscope. The most common of these is clear cell, which account for about 75 per cent of RCCs. Other subtypes include papillary, chromophobe, and collecting duct carcinoma.

Subtypes of renal cell carcinoma (RCC):
Conventional or clear cell RCC – this can also be called non-papillary RCC and accounts for 75 per cent of RCC cases. The cancer cells appear clear under the microscope and have large nuclei.
Papillary or chromophilic RCC accounts for about 10-15 per cent of RCC cases. The tumours have characteristic papillae or nodules on the surface.
Chromophobe RCC accounts for about 5 per cent of cases
Collecting duct carcinoma
Renal medullary carcinoma
Mucinous tubular and spindle-cell carcinoma
Renal translocation carcinomas
Unclassified RCC, the latter five of which together make up the remaining 5-10 per cent of RCC tumours

The word ‘cancer’ comes from the Latin for ‘crab’. Cancer is a group of diseases in which cells grow uncontrollably and have the ability to spread to another part of the body. There are more than 200 different cancers and each type of cancer has its own characteristics. When a cancer spreads to another part of the body it takes its own characteristics with it. So, for example, if a renal cancer spread to the liver, the tumour in the liver would both look and behave the same way as the original tumour. Cancer cells develop as a result of damage to DNA, the controlling mechanism for all the activities of the cells. This damage may be caused by a number of factors, including environmental, dietary and genetic. The body normally repairs damaged DNA, but in cancer cells this does not occur.

Cancers originate from a single cell. This cell divides and eventually forms a tumour. Tumours can be either malignant or benign.

Malignant tumours are cancerous tumours. The rate of cell division in cancer cells is greater than the rate of cell death, resulting in the growth of a tumour. Cancers do not always grow particularly fast. It is actually the lack of cell death in cancers that causes them to grow, rather than cell division being particularly fast. Cancerous tumours can originate in any of the common tissue types in the body.

Another important characteristic of cancer cells is their inability to recognise when they come into contact with different cell types. This means they are able to spread away into surrounding tissue and other areas of the body, via the blood stream or the lymphatic system (metastasise).

Benign tumours are not classified as cancers. The main difference between benign and malignant (cancerous) tumours is that benign tumours do not spread away from the site of origin.

The most common symptom is blood in the urine. Doctors call this haematuria. It may come and go and not every kidney cancer sufferer will have haematuria. Sometimes you won’t be able to see it, but it can still be detected by a urine test. Most people with blood in their urine do not have kidney cancer. It can be a sign of an infection, kidney stones, prostate problems or bladder cancer. However, it should always be investigated to find out what has caused it.

Most kidney cancers are too small to feel, but if you feel a lump or mass in the area of your kidneys you should tell your doctor straight away. You should also see your doctor about any persistent low back pain or pain, in your side between your ribs and hipbone (sometimes called the flank or loin). The sooner kidney cancer is detected, the easier it is to treat.

In the early stages of kidney cancer there may be no obvious symptoms. Many kidney cancers are found simply by chance when someone is being given a scan for another reason. More than half of adult kidney tumoursare detected when using an ultrasound scan to investigate symptoms, such as: high blood pressure, muscle wasting and weight loss, high temperature or fever, disorders affecting the nerves and muscles, inflammation, anaemia, abnormal liver function tests, and high levels of calcium in the blood (hypercalcaemia).

Sometimes abnormal red blood cell counts and high blood pressure, or hypertension, can be symptoms of kidney cancer. Some patients experience a condition called polycythaemia, or thickening of the blood, which can also be a symptom of kidney cancer. Symptoms of polycythaemia are a bad headache and redness of the skin.

The cancer may possibly have already spread to other organs for around a third of patients, such as the lungs, liver, brain and bones. These patients may experience symptoms of advanced kidney cancer, such as: a persistent cough, coughing-up blood (or haemoptysis), abnormal liver function tests, headaches and visual disturbances, or bone pain. You must see your doctor if you have any of these symptoms.

There are other symptoms, which can be more general and can also be caused by many other conditions, such as: weight loss, tiredness and running a persistent temperature and sweating heavily, especially at night.

Typical signs and symptoms of kidney cancer

• Persistent low back pain or pain in the side between the ribs and hipbone
• Blood in the urine, also called haematuria
• A lump or mass in the area of the kidneys
• Abnormal red blood cell counts
• High blood pressure or hypertension
• Thickening of the blood (polycythaemia)
• Tiredness
• Weight loss and/or loss of appetite
• Running a persistent temperature and sweating heavily, especially at night

With most cancers there is no direct cause and kidney cancer is no exception. For many people the cause of the cancer is never found. However, certain lifestyle factors can increase the risk of developing the disease;

The risk of developing kidney cancer is highest in people aged 45-75, and tails off in the late seventies.
An unhealthy diet and being overweight (obesity) increases the risk of developing kidney cancer.
Smoking may double the risk of developing kidney cancer for some people. The longer a person smokes and the more cigarettes they smoke, the greater the risk.
Genetic factors, such as a mutation in the von Hippel-Lindau gene, Birt-Hogg-Dubé syndrome, tuberous sclerosis and hereditary clear cell and papillary renal cell cancer, put people at greater risk of developing the disease.
Gender; men are twice as likely to suffer from kidney cancer than women.
Family history; people with a first degree relative (parent, sibling or child) with kidney cancer have about double the risk of developing kidney cancer themselves.
Certain medical conditions, such as high blood pressure (hypertension) and chronic kidney disease, especially people on long-term kidney dialysis, have a link to kidney cancer.
Previous thyroid cancer increases the risk of kidney cancer, possibly due to genetic changes that are common to both types of cancer.
Previous radiotherapy for testicular or cervical cancer (cancer of the neck of the womb) may slightly increase the risk of developing kidney cancer.
Long-term regular use of painkillers, such as ibuprofen, naproxen, phenacetin and celebrex is linked to kidney cancer.

A patient whose kidney cancer is confined to the kidney and has not spread into the lymph nodes or to other organs of the body, statistically stands the best chance of long-term survival. Some patients in this category will have their kidney and primary tumour removed by surgery and will never have any recurrence of the disease. In that sense they may be deemed ‘cured’. However, as with all cancers, there can never be complete certainty that cancer cells, carried through the lymphatic system and the blood, will not form into secondary tumours (metastases). Regular check-ups are essential for all kidney cancer patients, including those, who after surgery, are apparently free of disease.

According to medical statistics, patients in whom the disease has spread beyond the kidney are less likely to do well. If the disease has spread to one or more organs it is vitally important to have access to treatment that can stabilise or even eliminate secondary tumours. Some patients with secondary tumours respond well to further treatment (which is usually in the form of targeted therapy); others do not respond or respond only temporarily. We do not fully understand the reasons for this variation. One explanation is that kidney cancer cells vary in their level of aggressiveness from patient to patient. This relates mainly to the degree of abnormality of the cells, i.e. how different they are from normal cells. The higher the ‘grade’ of the kidney cancer cells, the more aggressive they are and the more likely they are to spread quickly. Some patients with secondary tumours live with their disease for a long time. A small proportion are ‘cured’ by treatment in the sense that their secondary tumours disappear completely. However even in these cases a recurrence can never be ruled out.

The kidneys are a pair of bean-shaped organs that are found high in the back of the abdominal cavity, just below the ribcage; one on either side of the spine. The right kidney is slightly lower than the left because of the position of the liver.

kidneys1

Adult kidneys measure approximately 10 to 12cm in length and 5 to 7 cm in width, and weigh approximately 150g.

If you were to cut a kidney in half you would see that it is divided into a dark outer area (the cortex) and an inner lighter-coloured area (the medulla). Within the medulla there are between 10 and 18 renal pyramids; triangular structures which have a striped appearance. They have this appearance because of the renal tubules and associated blood vessels. The renal cortex and the pyramids make up the renal parenchyma. It is here that approximately one million nephrons are the working centres of the kidneys.

Diagram of a Kidney

The kidneys filter the blood to remove waste products, which they convert into urine. Urine is carried from each kidney, through a tube called a ureter to the bladder, where it is stored. The ureter and blood vessels enter and exit the kidney through the renal hilum.

When you are ready to pass urine, it leaves the bladder through a tube called the urethra. The urethra opens immediately in front of the vagina in women and at the tip of the penis in men.

The kidneys have several other functions, such as blood pressure regulation, acid-base balance, toxin removal, red blood cell production, activation of vitamin D, water balance and electrolyte balance.

Surgery to remove the affected kidney (nephrectomy) is usually the first thing doctors consider and it can be a cure if the cancer is at an early stage. Even some more advanced cancers can be cured if all the cancer can be removed. However, removing a kidney is a major operation so you need to be fit enough to cope and recover afterwards. That’s why this treatment may not be possible for everyone. Surgery can also be used to remove metastases in some cases of advanced kidney cancer.

Partial nephrectomy

Removing part of the kidney containing the tumour is called a partial nephrectomy or kidney/nephron sparing surgery. The aim of this surgery is to remove the whole tumour while leaving as much normal tissue as possible. It means that some working kidney is left behind. For this reason, partial nephrectomy is carried out for people who only have one kidney, who have kidney disease, or who have tumours in both kidneys. Specialist surgeons now treat most early stage (stage 1) kidney tumours that are less than seven cm in size with partial nephrectomy, if possible.

Radical nephrectomy

During a radical nephrectomy the whole kidney and the surrounding fatty tissue, the adrenal gland, and nearby lymph nodes are usually removed, although the extent of a radical nephrectomy can vary between patients. You can live perfectly well with just one working kidney, but if both kidneys are removed because ofbilateral renal cell carcinoma, or because they are not working you will need dialysis for the rest of your life or a kidney transplant.

A radical nephrectomy may be carried out using open surgery, during which the surgeon usually makes a large incision or cuts below the lower ribs on the side of the affected kidney. The whole kidney and surrounding tissues are removed through this incision.

Keyhole (laparoscopic) and robotic surgery

Sometimes it is possible to use keyhole surgery or laparoscopic nephrectomy to remove the affected kidney, for which you will need a referral to a specialist urological surgeon with particular experience in laparoscopic kidney surgery. Laparoscopic surgery can also be used to carry out a partial nephrectomy.

Laparoscopic nephrectomy can be used when open surgery is not an option, for example patients with high blood pressure. The operation is carried out using several small incisions or cuts in the skin of the tummy (abdomen), rather than one large incision. A thin tube with a camera and a light at the end is inserted into the abdomen through one of the cuts. This instrument is called a laparoscope, and it enables the doctor to see inside your tummy. Other small instruments are inserted through the other cuts and used to remove the kidney or part of the kidney containing the tumour.

There may be some advantages to having keyhole surgery. For example, you may experience less pain after the operation, need a shorter stay in hospital and have smaller scars. However, keyhole surgery, like any operation, has some risks, so you should discuss the options with your specialist before surgery.

Assisted robotic surgery is a type of laparoscopic surgery in which a special machine or robot is used by the surgeon to help carry out the surgery. The surgeon has a 3D view of the inside of your tummy and the area can be magnified 10-12 times. Robotic surgery is only available at a few specialist centres in the UK.

After your operation

After the operation you will be given an intravenous drip of fluid and salts until you can eat and drink normally. Tubes will drain excess fluid from your wound to assist healing. These will be taken out before you go home. You will probably have a catheter fitted to drain your urine into a bag. This is usually taken out after a day or two.

Most people go home between four to ten days after their operation, depending on the type of surgery they had. The time it takes for you to feel fit enough to get back to leading a normal active life will vary. It may help to talk to your doctor or clinical nurse specialist (specialist nurse) about this.

A nephrectomy is a major operation and, as with any operation, there can be some risks, such as infection or blood clots (thrombosis) in the legs, arms or chest. If you are concerned by any of these, or if you have any symptoms of an infection (fever, feeling generally unwell) or a wound infection (redness, pain, swelling and oozing from the wound), please see your doctor.

Some people may find the scarring or changes to their body and how it works, difficult to deal with. Some may have concerns about the effects of the operation on relationships. You may need support to enable you to cope with such changes. Please talk to your doctor or nurse about how you feel and they will be able to put you in touch with somebody who can help.

You should get a follow up outpatient appointment to check your recovery six weeks after your surgery, where your consultant should discuss with you your prognosis and/or treatment options and follow-up schedule.

Follow-up after surgery

There are no guidelines for the method and timing of follow-up examinations after surgery. You will be followed-up for signs of recurrence of the cancer or spread of the cancer to other parts of the body. The frequency of follow-up visits and the tests that you receive during these visits will depend upon whether you experience any complications as a result of your operation, the function of your remaining kidney, and the risk of recurrence of the cancer or spread to other parts of the body. In general, you should have follow-up visits at six months and one year after your surgery, and then annually for at least five years. Patients at a low-risk of recurrence or cancer spread should have an ultrasound scan or chest X-ray, and blood tests to check kidney function at each visit. Patients at an intermediate or high-risk of recurrence or spread should have a CT scan and blood tests at each visit. These patients, or those with complications after their operation may be seen more frequently.

Other surgical treatments

Doctors have been researching less invasive ways of removing kidney tumours. These treatments can be used to remove small tumours (less than four cm in size) and for people who are unable to have surgery. They may also be used to treat people with multiple kidney tumours or tumours in both kidneys (bilateral disease).

Cryotherapy

Cryotherapy kills the cancer cells by freezing the tumour; however, there is only limited data to prove how effective this procedure is. The doctor inserts one or more fine needles or probes through the skin (percutaneous) and into the tumour. Argon gas or liquid nitrogen is passed through the needles under pressure to freeze the tumour. Regular CT scans are carried out during the procedure to ensure the needles are positioned correctly in the tumour and the entire tumour has been frozen. Cryotherapy is usually carried out under general anaesthetic, or sedation if a general anaesthetic is not suitable. Cryotherapy can also be carried out using keyhole surgery.

Complications or side-effects after cryotherapy include pain, infection and bleeding. A small number of people experience damage to the bowel and a blockage or damage to the ureter, the tube from the kidney to the bladder through which urine passes. However, in specialist centres that perform a lot of cryotherapy procedures, these complications are minimal.

National Institute for Health and Care Excellence (NICE) has issued guidance for percutaneous cryoablation; however, this procedure is only available at a few specialist centres in the UK. Further research is needed to compare the long-term outcomes of cryotherapy with those of other treatments for kidney cancer.

Radiofrequency ablation

Radiofrequency ablation (RFA) uses heat from an electric probe to destroy the tumour; however, there is only limited data to prove how effective this procedure is. Fine needles are inserted through the skin (percutaneous) and into the tumour. An electric current is passed through the needles to heat the cancer cells and destroy them. Regular CT or ultrasound scans are carried out during the procedure to ensure the needles are positioned correctly. RFA is usually carried out using local anaesthetic and sedation, and can also be carried out using keyhole surgery.

Complications or side-effects after RFA include pain, infection and bleeding. A small number of people experience damage to the kidney causing urine leakage and a narrowing of the ureter, making it difficult to pass urine. However, in specialist centres that have a lot of experience of RFA procedures, these complications are minimised.

National Institute for Health and Care Excellence (NICE) has issued guidance for percutaneous RFA; however, this procedure is only available at a few specialist centres in the UK. Further research is needed to compare the long-term outcomes of RFA with those of other treatments for kidney cancer.

High intensity focused ultrasound

High intensity focused ultrasound (HIFU) directs strong beams of sound at the tumour, which heats up the cancer cells and kills them. HIFU is only available in clinical trials for the treatment of kidney cancer in the UK, and research is ongoing to determine the effectiveness of this technique.

The main advantage of cryotherapy, RFA and HIFU is that they can be done using probes through the skin so you don’t have to have surgery. These approaches may be useful if your tumour is small, or when open or keyhole surgery to remove your kidney is not an option. However, these techniques are not widely-used and their success is yet to be proven. These treatments may be repeated if not successful first time.

If your surgeon is confident that all your cancer has been removed during surgery, you won’t need any further treatment. However, if there is concern that some cancer cells were left behind after surgery, you may need to have treatment with a drug called a targeted therapy or a course of radiotherapy. Your doctor or clinical nurse specialist (specialist nurse) will discuss this with you.

Surgical treatments:

Partial nephrectomy (open or keyhole)
Radical nephrectomy (open or keyhole)
Laparoscopic (keyhole) surgery or robot assisted surgery
Cryotherapy
Radiofrequency ablation (RFA)
High intensity focussed ultrasound (HIFU)

Transitional cell carcinoma (TCC), Upper urinary tract urothelial cancer, is cancer that starts in the upper urinary tract.

This is made up of the ureters and an area of the kidneys called the renal pelvis.  TCC arises from the inner lining of the urinary tract, called the transitional urothelium and accounts for the vast majority of bladder cancers and is not treated as a kidney cancer.

The treatment options for transitional cell carcinoma.

The treatment for transitional cell carcinoma (TCC) depends on several factors, such as the type of cancer, stage, and severity.

The most common treatment of TCC of the kidney is surgery. Depending on the stage and severity of the cancer, you may receive chemotherapy or (in rare cases) radiotherapy after surgery. If your cancer has progressed, you may be treated with chemotherapy or a combination of chemotherapy and radiotherapy.

Treatment options for TCC include:

• Surgery: Most people undergo radical nephroureterectomy, which removes the kidney, ureter, and a portion of their bladder and has the best chance of curing the disease. Some of the neighbouring lymph nodes and surrounding tissue may be removed. If the tumour is small and located in the ureter, your surgeon may be able to remove a portion of it in a procedure called segmental urethrectomy.
• Chemotherapy: If the cancer has spread to the surrounding tissue or lymph nodes, chemotherapy may be administered after the surgery. This lowers the likelihood of the cancer returning. Chemotherapy may also be recommended if the cancer is advanced, and you are not eligible for surgery.
• Regional chemotherapy: Regional chemotherapy involves inserting the tube through your urethra and into your ureter. Alternatively, a nephrostomy tube may be used.
• Radiotherapy: Radiotherapy is rarely used to treat TCC of the kidney or ureter. However, this treatment may be recommended if you are unable to have surgery or if your cancer has spread to adjacent tissue.

Symptoms of transitional cell carcinoma

The symptoms of transitional cell carcinoma (TCC) are similar to kidney cancer. The symptoms may vary, and patients with early-stage or low-grade TCC may have no symptoms at all. However, as the condition advances, the following symptoms may appear:

• New, persistent back pain or pelvic pain
• Blood in urine
• Frequent or painful urination
• Fatigue
• Unexplained weight loss

Have you had surgery, to remove the kidney and ureter called a nephroureterectomy and have you received a diagnosis of Transitional cell carcinoma (TCC)?

If the answer is Yes, you will be followed up under a bladder cancer surveillance pathway and not a kidney cancer pathway, as patients that have had kidney surgery with a histological confirmed diagnosis of Transitional cell carcinoma (TCC) and patients that have had a kidney removed with a histological confirmed diagnosis of Renal Cell cancer are managed under different treatment pathways, as the follow up surveillance pathway and the treatments given if the disease progresses are very different to a patient that has had a kidney removed with a histological confirmed diagnosis of Renal Cell cancer (Kidney cancer).

Follow up following a diagnosis of TCC

You will have follow -up appointments after surgery for transitional cell cancer of the kidney or ureter. Your urology doctor will look for signs of the cancer coming back or spreading so that if found can be treated early.

Your urology doctor will tell you exactly how and how often they will keep a check on you.

As part of your follow up you have regular tests to look inside your bladder (cystoscopies). This is because transitional cell cancers can sometimes come back in the bladder.

Kidney Cancer UK offers advice and support to those affected by kidney cancer but not transitional cell cancer (TCC). Below links to the charities that can provide you with the correct advice and support.

Action Bladder Cancer UK 

 Fight Bladder Cancer

Fight Bladder Cancer closed Facebook Support Group

Von Hippel-Lindau syndrome (VHL) is a rare inherited genetic condition which is characterised by the growth of tumours and fluid filled sacs (cysts). VHL syndrome is caused by a genetic mutation in the VHL gene, which is a Tumour suppresser gene.

A variety of tumours (some benign and some malignant) are commonly associated with VHL syndrome. Between 28-45% of VHL patients develop kidney cancer. Clear cell renal cell carcinoma is the only type of kidney cancer associated with VHL and it can metastasise and become aggressive.

Tumours made from newly formed blood vessels (called haemangioblastomas) are common in patients with VHL. The places most often affected are the brain (particularly a part of the brain called the cerebellum), the spinal cord, and the retina (the lining of the back of the eye). These tumours are non-cancerous (benign), but sometimes can be quite serious because of where they develop.

More information on von Hippel-Lindau syndrome can be found on the VHL Family Alliance website.

Kidney cancer rarely affects children, and about 45-50 paediatric cases are diagnosed in the UK each year. About 75% of childhood kidney cancer occurs in the under-fives. The most common paediatric kidney cancer is Wilms’ tumour, which account for up to 95 per centof childhood kidney cancers. Others include the following very rare types of kidney cancer;

Renal cell carcinoma is the most common form of kidney cancer among adults, but it is rare in children younger than 15 years of age. However, it is more common in adolescents between 15 and 19 years of age. Renal cell carcinomas can spread to the lungs, bones, liver, and lymph nodes. Renal cell carcinoma in children is sometimes associated with genetic conditions, such as von Hippel-Lindau disease (an inherited condition that causes abnormal growth of blood vessels), tuberous sclerosis (an inherited disease marked by noncancerous fatty cysts in the kidney), neuroblastoma and sickle cell disease.
Clear cell sarcoma of the kidney is a very rare type of kidney tumour that affects the supporting tissues in the kidney, and may spread to the lungs, bones, brain, and soft tissue. It is most common in infants and young children aged one to four years.
Rhabdoid tumour of the kidney is a type of cancer that occurs mostly in infants and very young children. It grows and spreads quickly, often to the lungs and brain. A fault (mutation) in a gene called INI1 may increase the risk of developing rhabdoid tumours in the kidney.
Ewing sarcoma (neuroepithelial tumour) of the kidney is rare and usually occurs in adolescents. These tumours grow and spread to other parts of the body quickly.
Desmoplastic small round cell tumour of the kidney is a rare soft tissue sarcoma that most often affects boys, and which may develop and spread in the tummy (abdomen), pelvis or tissues around the testes. These tumours may also spread to the lungs and other parts of the body.
Synovial sarcoma is most common in adolescents and grows and spreads quickly.
Anaplastic sarcoma is most common in children younger than 15 years and often spreads to the lungs, liver, or bones.
Cystic partially differentiated nephroblastoma is a very rare type of Wilms’ tumour made up of cysts.
Mesoblastic nephroma is a non-cancerous (benign) tumour of the kidney that is usually diagnosed within the first year of life, and can usually be cured with surgery alone. One type of mesoblastic nephroma may appear on an ultrasound examination before birth or may occur within the first three months after the child is born.
Multilocular cystic nephromas are non-cancerous (benign) tumours made up of cysts, which can occur in one or both kidneys. Children with this type of tumour could also have pleuropulmonary blastomas in the lungs.
These very rare kidney tumours are often treated in a similar way to unfavourable (high-risk) Wilms’ tumour, with surgery, chemotherapy and sometimes radiotherapy (see below). Mesoblastic and multilocular cystic nephromas, however, are benign tumours that can be cured using surgery alone and no other treatment.

Wilms’ tumour

Wilms’ tumour (sometimes called nephroblastoma) is a very rare type of kidney cancer, which mainly affects young children under five years of age. It is named after a German doctor, Dr Max Wilms (1867-1918), who first described it. About 40 children are diagnosed with Wilms’ tumour in the UK each year, and it is curable in around 90 per cent of cases. The vast majority of Wilms’ tumours affect only one kidney (unilateral), but in about seven per cent of cases, both kidneys (bilateral) are affected.

A nephroblastoma is abnormal tissue, which grows on the outer part of one or both kidneys. It is thought that nephroblastomas originate from specialised cells in the developing embryo known as metanephric blastema, which are involved in the development of the child’s kidneys while they are still in the womb. These cells usually disappear at birth, but in some children they can still be found on the kidneys after birth, and are called nephrogenic rests. Children with this condition are at risk of developing a type of Wilms’ tumour.

Wilms’ tumour can be categorised depending on how the tumour cells appear under the microscope into two main types; favourable histology or unfavourable histology. Tumour cells with unfavourable histology look very large and not like normal kidney cells under the microscope. They are called anaplastic, and the cancer is less likely to be cured if anaplastic cells are widespread in the tumour. However, about 95 per cent of Wilms’ tumours have favourable histology and a high chance of a cure.

Causes and risk factors for Wilms’ tumour

The causes of Wilms’ tumour in most children are unknown. However, several risk factors have been identified. Anything that increases the risk of getting a disease is called a risk factor. However, having a risk factor does not mean that you or your child will necessarily get cancer.

In a few cases, it is thought that Wilms’ tumour may be part of a genetic syndrome that affects the growth and development of the child. A genetic syndrome is a set of symptoms or conditions that occur together, and is usually caused by abnormal genes. Certain birth defects can also increase a child’s risk of developing Wilms’ tumour. The following genetic syndromes have been linked to Wilms’ tumour:

Wilms’ tumour, Aniridia, abnormal Genitourinary system and mental Retardation (WAGR syndrome) is a combination of abnormalities affecting the coloured part of the eye (the iris) where the iris is partially or totally absent (aniridia), the genitourinary tract (the kidneys, urinary tract, penis, scrotum, testicles, clitoris or ovaries), and possibly the brain causing learning difficulties. Often children present with a milder form of WAGR with only some of these abnormalities.
Beckwith-Wiedemann syndrome is a condition where children have larger than normal internal organs, often an enlarged tongue, and sometimes one arm or leg may be bigger than the other.
Hemihypertrophy is a condition in which children have one side of the body slightly larger than the other. Hemihypertrophy is linked with Wilms’ tumour risk when it forms part of Beckwith-Wiedemann syndrome. However, most patients with isolated hemihypertrophy are not at increased risk of Wilms’ tumour development.
Denys-Drash syndrome is a condition where boys do not develop normal male genitalia (penis, scrotum or testicles), and can be mistaken for girls. Their kidneys do not develop properly and eventually stop working. A Wilms’ tumour can grow in the damaged kidney.
There is also a slight risk due to a family history for Wilms’ tumour; between one and two out of every 100 children (one to two per cent) with Wilms’ tumour have another family member with the disease due to the inheritance of an abnormal gene from their parents.

Signs and symptoms of Wilms’ tumour

Wilms’ tumours can be very big when they are discovered due to the fact that the tumour is usually painless. In some cases the tumour can be much bigger than the kidney itself, although it is unusual for the tumour to have spread to other parts of the body from these large tumours.

The most common symptom is a painless swelling of the tummy. Other less common symptoms are listed below. Other conditions may cause the same symptoms, and a doctor should be consulted if you notice any of the following problems:

Painless swelling or lump in the tummy (abdomen). This is the most common symptom of Wilms’ tumour. Occasionally, the tumour may bleed slightly causing irritation and pain in the area surrounding the kidney.
Blood is found in the urine in around 15-20 per cent of children with Wilms’ tumour.
Raised blood pressure.
High temperature or fever for no known reason.
Loss of appetite.
Weight loss.
Upset stomach and/or vomiting.
Shortness of breath and/or persistent cough is a symptom if the cancer has spread to the lungs.
Tests for childhood kidney cancer

The following tests and investigations may be used for the detection of childhood kidney cancer, including Wilms’ tumour:

Physical examination of the body to check your child’s general health and to look for any lumps or swellings. A medical history of your child’s past illnesses and treatments will also be taken. This will most likely take place at your GP surgery and be repeated in hospital if your child is referred for further tests.
Blood and urine samples will be taken to check your child’s kidney function and general health. They can take place in your GP surgery.
Abdominal ultrasound scan and a CT scan are often done at the hospital to help diagnose the tumour and to assess its growth and spread. Occasionally scans of the chest and liver may be taken to check the spread of the disease. The information obtained from these scans is used to stage the progress of the disease (see below).
Biopsy of the tumour is taken from most children to confirm the diagnosis made using the scans. There are two types of biopsy; during a needle biopsy cells are removed from the tumour using a needle inserted through the skin and into the tumour under local anaesthetic. During a tissue biopsy a piece of tumour is removed during a small operation under general anaesthetic. Sometimes, the surgeon will remove the entire tumour during this operation. The cell and tissue samples are sent to a pathologist, who examines them under a microscope to confirm the tumour as having favourable or unfavourable histology.
Staging of Wilms’ tumour

Wilms’ tumours can be categorised into stages, which describe the size and spread of the tumour. The information used to stage Wilms’ tumour is obtained from ultrasound and CT scans, and is used to help doctors decide on the most appropriate treatment. Often, the stage of your child’s tumour might not be known until after surgery, since most children have chemotherapy to shrink the tumour before surgery to remove it. A commonly-used staging system for Wilms’ tumours is as follows:

Stage 1 The tumour affects only one kidney, is contained within 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 it is still possible to remove it completely with surgery.
Stage 3 The tumour has spread beyond the kidney, or the tumour has spread to the lymph nodes (glands), or it has not been completely removed by surgery.
Stage 4 The tumour has spread to other parts of the body such as the lungs, liver, brain or bone to form metastases or secondary tumours.
Stage 5 There are tumours in both kidneys (bilateral Wilms’ tumour).
Recurrent cancer. The tumour has come back after initial treatment.
Treatment for Wilms’ tumour

Treatment for Wilms’ tumours will depend on the stage and spread of the disease, as described above, in addition to the histology of the tumour i.e. whether it is has favourable or unfavourable histology. Those children with a Wilms’ tumour of unfavourable histology or high-risk tumour will have stronger treatment.

Surgery All children with Wilms’ tumour will have surgery. In most cases, a biopsy will be taken to confirm the diagnosis and to determine the histology of the tumour as favourable (standard or low risk) or unfavourable (high risk). The tumour will be removed by surgery, usually following a course of chemotherapy to shrink the tumour. Chemotherapy is not given to infants less than six months old. In most cases, the whole kidney is taken out (a nephrectomy), but occasionally only part of the kidney is removed (partial nephrectomy). In the case of bilateral Wilms’ tumour, surgeons try to ensure that as much healthy kidney remains as possible after removal of the cancerous tissue.
Chemotherapy is usually given both before surgery to shrink the tumour and make it easier to remove (neo-adjuvant chemotherapy), and after surgery to kill off any cancer cells left behind after surgery (adjuvant chemotherapy), thereby reducing the risk of the tumour coming back or recurring. Chemotherapy is the use of drugs, which are toxic to cancer cells (cytotoxic drugs) but relatively harmless to normal cells. These drugs stop the cancer cells from growing and can be given alone or in combination with other cytotoxic drugs, depending on the stage and histology of the tumour. Chemotherapy is usually given by means of an injection into a vein, or via a drip, which requires the use of a catheter placed in a large vein, usually in the neck. The catheter is put in place during a small operation, and usually stays throughout the course of treatment.
Radiotherapy may also be given, depending on the stage and histology of the tumour. Not all children need radiotherapy. Radiotherapy involves the use of high-energy radiation to destroy cancerous cells. It can be directed to the site of the affected kidney, thereby causing the least amount of harm to surrounding normal tissue. Sometimes, radiotherapy is used to treat the whole tummy area or the lungs, if the cancer has spread to the lungs. However, the use of radiotherapy depends on how well the cancer responds to initial chemotherapy, and it is not always needed.
Chemotherapy and radiotherapy are used to treat Wilms’ tumour that has spread to other organs and tissues (metastasised) i.e. stage 4 Wilms’ tumour.

Chemotherapy and radiotherapy often cause side-effects, which your doctor will discuss with you prior to treatment. These include feeling sick (nausea) and being sick (vomiting), diarrhoea, hair loss, tiredness, bruising and bleeding, and an increased risk of infection. Additionally, some children may experience late side-effects many years later, including reduced bone growth, a change in heart and lung function, infertility, and a slightly increased risk of developing cancer again in later life.

Axitinib is targeted therapy drug which is used for advanced kidney cancer. It is a tyrosine kinase inhibitor (TKI), which blocks the effects of the tyrosine kinases involved in new blood vessel growth that is essential for cancer cells to divide and grow. These treatments starve the tumour by stopping the development of a new blood supply (angiogenesis). Treatments that interfere with the development of a blood supply are called anti-angiogenic agents.

Axitinib comes as a tablet to take by mouth. Initially a 5mg tablet is taken twice daily with or without food, about 12 hours apart. The dosage is then increased or decreased between a range of 2-10mg twice daily depending on safety and tolerability.

The most common side effects include fatigue, stomach upsets from diarrhoea to nausea and vomiting, skin discoloration, red and blistered hands and feet, sore mouth, an increase in blood pressure, and loss of taste and appetite. Many of these side-effects can be controlled with medication and they do not affect everyone.

In February 2015 Axitinib was recommended by NICE as the second-line treatment option for advanced renal cell carcinoma if prior systemic treatment has failed.

Sorafenib is a targeted therapy drug used to try to shrink or control advanced kidney cancer and help people to live longer.

Sorafenib is a tyrosine kinase inhibitor, which blocks the effects of the tyrosine kinases involved in new blood vessel growth that is essential for cancer cells to divide and grow. These treatments starve the tumour by stopping the development of a new blood supply (angiogenesis). Treatments that interfere with the development of a blood supply are called anti-angiogenic agents.

Sorafenib is usually taken as two 200mg tablets twice-a-day for as long as the treatment is helping.

The most common side effects include fatigue, stomach upsets from diarrhoea to nausea and vomiting, skin discoloration, red and blistered hands and feet, sore mouth, an increase in blood pressure, and loss of taste and appetite. Many of these side-effects can be controlled with medication and they do not affect everyone.

Although sorafenib has been licensed for the treatment of people with advanced kidney cancer, the National Institute for Health and Clinical Excellence (NICE) has issued guidance that recommends sorafenib is NOT used as a first-line or second-line treatment option in NHS hospitals. Instead, sunitinib and pazopanib (both tyrosine kinase inhibitors) are recommended by NICE as first-line treatment options. Axitinib is recommended by NICE as the second-line treatment option for advanced renal cell carcinoma if prior systemic treatment has failed.

Sunitinib is a targeted therapy drug used to try to shrink or control advanced kidney cancer and help people to live longer.

Sunitinib is a tyrosine kinase inhibitor (TKI), which blocks the effects of the tyrosine kinases involved in new blood vessel growth that is essential for cancer cells to divide and grow. These treatments starve the tumour by stopping the development of a new blood supply (angiogenesis). Treatments that interfere with the development of a blood supply are called anti-angiogenic agents.

Sunitinib comes as a capsule, which is swallowed. You usually take one 50 mg capsule a day for four weeks, followed by two weeks off.

The most common side-effects include fatigue, stomach upsets (diarrhoea, nausea and vomiting), skin discoloration, red and blistered hands and feet, sore mouth, an increase in blood pressure, and loss of taste and appetite. Many of these side-effects can be controlled with medication and they do not affect everyone.

In the UK, sunitinib is used as a first-line treatment for advanced renal cell carcinoma. The National Institute for Health and Clinical Excellence (NICE) has issued guidance that recommends only sunitinib and pazopanib as first-line treatment options in NHS hospitals. Axitinib is recommended by NICE as the second-line treatment option for advanced renal cell carcinoma if prior systemic treatment has failed.

Temsirolimus is a targeted therapy drug treatment called an mTOR inhibitor. It blocks the effects of a particular protein called mTOR that is often over active in cancer cells and makes the cells divide and grow. Temsirolimus also stops the cancer from making blood vessels, which the cells need to be able to grow. It is called an anti-angiogenesis treatment. So temsirolimus helps to stop the cancer growing or may slow the growth. Temsirolimus is a treatment for advanced kidney cancer and for mantle cell lymphoma. Side-effects to mTOR inhibitors are similar to those for Tyrosine Kinase inhibitors.

Temsirolimus is licensed for the treatment of advanced kidney cancer in the UK. However, it has not been recommended by The National Institute for Health and Clinical Excellence (NICE) for use on the NHS. Funding for this treatment can be obtained through government funding schemes, such as the Cancer Drugs Fund, or you may have treatment with mTOR inhibitors as part of a clinical trial.

Like sunitinib, pazopanib is a targeted therapy drug. Targeted therapy drugs are used to try to shrink or control advanced kidney cancer and help people to live longer. Pazopanib is a tyrosine kinase inhibitor (TKI), which blocks the effects of the tyrosine kinases involved in new blood vessel growth that is essential for cancer cells to divide and grow. These treatments starve the tumour by stopping the development of a new blood supply (angiogenesis). Treatments that interfere with the development of a blood supply are called anti-angiogenic agents.

Pazopanib comes as a 800 mg tablet to take by mouth. It is usually taken on an empty stomach once-a-day, at least one hour before or two hours after a meal. Unlike sunitinib, pazopanib is taken continuously.

The most common side-effects include fatigue, stomach upsets (diarrhoea, nausea and vomiting), skin and hair discoloration, hair loss, mild stomach pain, indigestion, loss of taste and appetite, weakness and weight loss. Many of these side effects can be controlled with medication and they do not affect everyone.

In the UK, pazopanib is used as a first-line treatment for advanced renal cell carcinoma. The National Institute for Health and Clinical Excellence (NICE) has issued guidance that recommends only sunitinib and pazopanib as first-line treatment options in NHS hospitals. Axitinib is recommended by NICE as the second-line treatment option for advanced renal cell carcinoma if prior systemic treatment has failed.

Everolimus is targeted therapy drug which is used for advanced kidney cancer. It belongs to a group of protein kinase inhibitors, called mTOR inhibitors, which block the effects of a particular protein called mTOR that is often over active in cancer cells and makes the cells divide and grow. Everolimus is an oral treatment and is taken once daily. mTOR inhibitors are treatments for advanced kidney cancer that has come back during or after treatment. Side-effects to mTOR inhibitors are similar to those for Tyrosine Kinase inhibitors.

Everolimus is licensed for the treatment of advanced kidney cancer in the UK; however, it has not been recommended by NICE for use of the NHS. Funding for this treatment can be obtained through government funding schemes, such as the Cancer Drugs Fund, or you may have treatment with mTOR inhibitors as part of a clinical trial.

Pembrolizumab (pronounced pem-bro-lih-zoo-mab) is a type of immunotherapy. It is also known by its brand name, Keytruda. 

You might have it as a treatment by itself or in combination with another treatment for a number of different cancer types.