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What is cancer?
The body is made up of trillions of living cells. Normal body cells grow, divide to make new
cells, and die in an orderly fashion. During the early years of a persons life, normal cells
divide faster to allow the person to grow. After the person becomes an adult, most cells
divide only to replace worn-out or dying cells or to repair injuries.
Cancer begins when cells in a part of the body start to grow out of control. There are many
kinds of cancer, but they all start because of out-of-control growth of abnormal cells.
Cancer cell growth is different from normal cell growth. Instead of dying, cancer cells
continue to grow and form new, abnormal cells. In most cases the cancer cells form a tumor.
Cancer cells can also invade (grow into) other tissues, something that normal cells cannot do.
Growing out of control and invading other tissues are what makes a cell a cancer cell.
Cells become cancer cells because of damage to DNA. DNA is in every cell and directs all its
actions. In a normal cell, when DNA gets damaged the cell either repairs the damage or the
cell dies. In cancer cells, the damaged DNA is not repaired, but the cell doesnt die like it
should. Instead, this cell goes on making new cells that the body does not need. These new
cells will all have the same damaged DNA as the first cell does.
People can inherit damaged DNA, but often the DNA damage is caused by mistakes that
happen while the normal cell is reproducing or by something in our environment. In adults,
sometimes the cause of the DNA damage is something obvious, like cigarette smoking. But
often no clear cause is found.
Cancer cells often travel to other parts of the body, where they begin to grow and form new
tumors that replace normal tissue. This process is called metastasis. It happens when the
cancer cells get into the bloodstream or lymph vessels of our body.
Different types of cancer can behave very differently. They grow at different rates and
respond to different treatments. That is why people with cancer need treatment that is aimed
at their particular kind of cancer.
Not all tumors are cancerous. Tumors that arent cancer are called benign. Benign tumors can
cause problems they can grow very large and press on healthy organs and tissues. But they
cannot grow into (invade) other tissues. Because they cant invade, they also cant spread to
other parts of the body (metastasize). These tumors are almost never life threatening.
The kidneys main job is to filter the blood and rid the body of excess water, salt, and waste
products. The filtered products and extra water are changed into urine. Urine leaves the
kidneys through long, slender tubes called ureters that connect to the bladder. Urine flows
down the ureters into the bladder, and is stored there until the person urinates.
The kidneys also have other jobs:
They help control blood pressure by making a hormone called renin.
They help make sure the body has enough red blood cells. They do this by making a
hormone called erythropoietin, which tells the bone marrow to make more red blood
cells.
Our kidneys are important, but we actually need less than one complete kidney to do all of its
basic functions. Tens of thousands of people in the United States are living normal, healthy
lives with just one kidney.
Wilms tumors
Wilms tumors are the most common cancers in children that start in the kidneys. Most Wilms
tumors are unilateral, which means they affect only one kidney. Most often there is only one
tumor, but 5% to 10% of children with Wilms tumors have more than one tumor in the same
kidney. About 5% of children with Wilms tumors have bilateral disease (cancer in both
kidneys).
Wilms tumors often become quite large before they are noticed. The average newly found
Wilms tumor is many times larger than the kidney in which it started. Most tumors are found
before they have spread (metastasized) to other organs.
Even though a doctor might think a child has a cancer such as Wilms tumor based on a
physical exam or imaging tests, they cannot be certain until a sample of the tumor is looked
at under a microscope.
Age
Wilms tumors are most common in young children, with the average age being about 3 to 4
years. They are less common in older children, and are rare in adults.
Race
In the United States, the risk of Wilms tumor is slightly higher in African-American children
than in white children and is lowest among Asian-American children. The reason for this is
not known.
Gender
The risk of Wilms tumor is slightly higher in girls than in boys.
WAGR syndrome
WAGR stands for the first letters of the physical and mental problems linked with this
syndrome (although not all children have all of them):
Wilms tumor
Aniridia (complete or partial lack of the iris [colored area] of the eyes)
Genitourinary tract abnormalities (defects of the kidneys, urinary tract, penis, scrotum,
clitoris, testicles, or ovaries)
Mental Retardation
Children with this syndrome have about a 30% to 50% chance of having a Wilms tumor. The
cells in children with WAGR syndrome are missing part of chromosome 11, where the WTJ
gene is normally found. Children with WAGR tend to get Wilms tumors at an earlier age and
often have bilateral disease (tumors in both kidneys).
Beckwith-Wiedemann syndrome
Children with this syndrome tend to be big for their age. They also have larger than normal
internal organs and often have an enlarged tongue. They may have an oversized arm and/or
leg on one side of the body (hemihypertrophy), as well as other medical problems. They have
about a 5% risk of having Wilms tumors (or, less often, other cancers that develop during
childhood). This syndrome is also caused by a defect in chromosome 11.
Denys-Drash syndrome
This rare syndrome has been linked to changes (mutations) in the WTJ gene. In this syndrome
the kidneys become diseased and stop working when the child is very young. Wilms tumors
usually develop in the diseased kidneys. The reproductive organs do not develop normally,
which in boys may cause them to be mistaken for girls. Because the risk of
Wilms tumors is very high, doctors often advise removing the kidneys soon after this
syndrome is diagnosed.
Other syndromes
Less often, Wilms tumor has been linked to other syndromes, including:
Penman syndrome
Sotos syndrome
Simpson-Golabi-Behmel syndrome
Bloom syndrome
Li-Fraumeni syndrome
Frasier syndrome
Trisomy 18
Aniridia (complete or partial lack of the iris [colored area] of the eyes)
Hemihypertrophy (an oversized arm and/or leg on one side of the body)
Cryptorchidism (failure of the testicles to descend into the scrotum) in boys
Hypospadias (defect in boys where the urinary opening is on the underside of the penis)
cells that makes up our genes the instructions for how our cells function.
Some genes control when our cells grow, divide into new cells, and die. Certain genes that
help cells grow, divide, or stay alive are called oncogenes. Others that slow down cell
division or cause cells to die at the right time are called tumor suppressor genes. Cancers can
be caused by DNA changes that turn on oncogenes or turn off tumor suppressor genes. These
gene changes can be inherited from a parent (as is sometimes the case with childhood
cancers), or they may happen during a persons lifetime as cells in the body divide to make
new cells.
Changes in certain genes increase the chance that some kidney cells will remain in an early
form and turn into a Wilms tumor.
For example, a small number of Wilms tumors are caused by changes (mutations) in or loss
of the WT] or WT2 genes, which are tumor suppressor genes found on chromosome 11.
Changes in these genes and some other genes on chromosome 11 can lead to overgrowth of
certain body tissues. This may explain why other growth abnormalities, like some of those
described in the section What are the risk factors for Wilms tumor? are sometimes found
together with Wilms tumors.
Sometimes these gene changes are passed on from a parent to a child, but most Wilms
tumors do not seem to be caused by inherited gene mutations. Instead, they seem to be the
result of gene changes that occur early in a childs life, perhaps even before birth.
In a small number of Wilms tumors there is a change in a tumor suppressor gene known as
WTX, which is found on the X chromosome. Another gene that is sometimes altered in Wilms
tumor cells is known as CTNNB]. Its not clear exactly what causes these genes to be altered.
Because the genes described above are not altered in all cases of Wilms tumors, there must
be changes in other genes that have not yet been found. In many cases, more than one gene
change is involved.
Researchers now understand some of the gene changes that can occur in Wilms tumors, but
its still not clear what might cause these changes. Some gene changes may be inherited, but
most Wilms tumors are not the result of known inherited syndromes. Other gene changes
may just be random events that sometimes happen inside a cell, without having an outside
cause. There are no known lifestyle-related or environmental causes of Wilms tumors, so it is
important to remember that there is nothing these children or their parents could have done to
prevent these cancers.
Can Wilms tumor be prevented?
The risk of many adult cancers can be reduced with certain lifestyle changes (such as staying
at a healthy weight or quitting smoking), but at this time there are no known ways to prevent
most cancers in children.
The only known risk factors for Wilms tumors (age, race, gender, and certain inherited
conditions) cannot be changed. There are no known lifestyle-related or environmental causes
of Wilms tumors, so at this time there is no way to protect against most of these cancers.
Experts think these cancers come from cells that were in the fetus but failed to develop into
mature kidney cells. This doesnt seem to be caused by anything that a mother could avoid.
In some very rare cases, such as in children with Denys-Drash syndrome who are almost
certain to develop Wilms tumors, doctors may recommend removing the kidneys at a very
young age (with a donor kidney transplant later on) to prevent tumors from developing.
Fever
Nausea
Loss of appetite
Shortness of breath
Constipation
Blood in the urine
Wilms tumors can also sometimes cause high blood pressure. This does not usually cause
symptoms on its own, but in rare cases it can get high enough to cause problems such as
bleeding inside the eye or even a change in consciousness.
Many of the signs and symptoms above are more likely to be caused by something other than
a kidney tumor. Still, if your child has any of these symptoms, check with your childs doctor
so that the cause can be found and treated, if needed.
To help find out if there is a tumor in the kidney(s), and if so, if it is likely to be a Wilms
tumor
To learn how far the tumor may have spread, both within the kidney and to other parts of
the body
Ultrasound (sonogram)
This is often the first imaging test done if the doctor suspects your child has a Wilms tumor
because its easy to have, it does not use radiation, and it gives the doctor a good view of the
kidneys and the other organs in the abdomen.
Ultrasound uses sound waves to create images of internal organs. For this test, your child lies
on a table while a small wand called a transducer is placed on the skin (which is first
lubricated with a gel) over the belly. It gives off sound waves and picks up the echoes as they
bounce off the kidney. The echoes are converted by a computer into a black and white image
on a screen.
The echo patterns made by most kidney tumors look different from those of normal kidney
tissue. Different echo patterns also can help doctors tell some types of cancerous and non-
cancerous kidney tumors apart from one another.
Ultrasound is also very useful when looking for tumor thrombus (tumor growing into the
main veins coming out of the kidney). This helps in planning for surgery, if it is needed.
The test is not usually painful, but it might cause some discomfort if the transducer is pressed
down hard on the abdomen.
a narrow tube, which is confining and can be distressing. Newer, more open MRI machines
may help with this, but the test still requires staying still for long periods of time. The MRI
machine also makes loud buzzing and clicking noises that your child may find disturbing.
Younger children may be given medicine to help keep them calm or even asleep during the
test.
Chest x-ray
Chest x-rays may be done to look for any spread of Wilms tumor to the lungs, as well as to
have a baseline view of the lungs to compare with other x-rays that might be done in the
future. If a CT scan of the chest is done, this test is not needed.
Bone scan
Bone scans can help show if cancer has spread to bones. Doctors dont usually order this test
unless they think your child has a type of Wilms tumor that is likely to spread.
For this test, a small amount of low-level radioactive material is injected into a vein
(intravenously, or IV). (The amount of radioactivity used is very low and will pass out of the
body within a day or so.) The substance settles in areas of damaged bone throughout the
skeleton over the course of a couple of hours. Your child then lies on a table for about 30
minutes while a special camera detects the radioactivity and creates a picture of the skeleton.
Younger children may be given medicine to help keep them calm or even asleep during the
test.
Areas of active bone changes will appear as hot spots on the skeleton that is, they attract
the radioactivity. These areas may suggest the presence of cancer, but other bone diseases
can also cause the same pattern. To help tell these apart, other tests such as plain x-rays or
MRI scans of the bone might be needed.
Lab tests
Lab tests might be done to check urine and blood samples if your childs doctor suspects a
kidney problem. They may also be done after a diagnosis of Wilms tumor has been made.
A urine sample may be tested (urinalysis) to look for blood and other substances in the urine
to see if there are problems with the kidneys. The urine may also be tested for substances
called catecholamines. This is done to make sure your child doesnt have another kind of
tumor called neuroblastoma. (Neuroblastomas often start in the adrenal gland, which lies just
above the kidney.)
Blood tests are not used to find Wilms tumors, but they can sometimes show if a child has
kidney problems. They can also be done to check a childs general health (especially before
surgery) and to look for side effects during treatment such as chemotherapy. These may
include tests to count the number of white blood cells, red blood cells, and blood platelets,
and tests to measure certain chemicals and salts in the blood that give clues about how well
the kidneys and liver are working.
Kidney biopsy/surgery
Most of the time, imaging tests can give doctors enough information to decide if a child
probably has a Wilms tumor, and therefore if surgery should be done. But the actual
diagnosis of Wilms tumor is made when a sample of the tumor is removed and looked at
under a microscope. The cells in Wilms tumors have a distinct appearance when looked at
this way. Doctors also look at the sample to determine the histology of the Wilms tumor
(favorable or unfavorable), as was described in the section What is Wilms tumor?
In most cases, the sample is removed during surgery to treat the tumor (see the Surgery
section). Sometimes if the doctors are less certain about the diagnosis or if they are not sure
the tumor can be removed completely, a sample of the tumor may be taken during a biopsy as
a separate procedure done before surgery. The biopsy may be done either as a type of surgery
or using a long, hollow needle thats inserted through the skin and into the tumor.
See Testing Biopsy and Cytology Specimens for Cancer to learn more about different types of
biopsies, how the tissue is used in the lab for disease diagnosis, and what the results will tell
you.
Stage I
The tumor was contained within one kidney and was completely removed by surgery. The
tissue layer surrounding the kidney (the renal capsule) was not broken during surgery. The
cancer had not grown into blood vessels in or next to the kidney. The tumor was not biopsied
before surgery to remove it.
About 40% to 45% of all Wilms tumors are stage I.
Stage II
The tumor has grown beyond the kidney, either into nearby fatty tissue or into blood vessels
in or near the kidney, but it was completely removed by surgery without any apparent cancer
left behind. Lymph nodes do not contain cancer. The tumor was not biopsied before surgery.
About 20% of all Wilms tumors are stage II.
Stage III
This stage refers to Wilms tumors that may not have been completely removed. The cancer
remaining after surgery is limited to the abdomen (belly). One or more of the following
features may be present:
The cancer has spread to lymph nodes (bean-sized collections of immune cells) in the
abdomen or pelvis but not to more distant lymph nodes, such as those inside the chest.
The cancer has invaded nearby vital structures so the surgeon could not remove it
completely.
Deposits of tumor (tumor implants) are found along the inner lining of the abdominal
space.
Cancer cells are found at the edge of the sample removed by surgery, indicating that
some of the cancer still remains after surgery.
Cancer cells spilled into the abdominal space before or during surgery.
The tumor was removed in more than one piece for example, the tumor was in the
kidney and in the nearby adrenal gland, which was removed separately.
A biopsy of the tumor was done before it was removed with surgery.
About 20% to 25% of all Wilms tumors are stage III.
Stage IV
The cancer has spread through the blood to organs away from the kidneys such as the lungs,
liver, brain, or bone, or to lymph nodes far away from the kidneys.
About 10% of all Wilms tumors are stage IV.
Stage V
Tumors are found in both kidneys at diagnosis.
About 5% of all Wilms tumors are stage V.
Survival rates for Wilms tumor by stage and histology
Survival rates are often used by doctors as a standard way of discussing a persons prognosis
(outlook). Some parents may want to know the survival statistics for children in similar
situations, while others may not find the numbers helpful, or may even not want to know
them. If you would rather not read about survival rates, please skip to the next section.
The 4-year survival rate refers to the percentage of children who live at least 4 years after
their cancer is diagnosed. Of course, many children live much longer than 4 years (and many
are cured).
In order to get 4-year survival rates, doctors have to look at children who were treated at least
4 years ago. Improvements in treatment since then may result in a better outlook for children
now being diagnosed with Wilms tumors.
The survival rates below are based on the results of the National Wilms Tumor Studies,
which included most of the children treated in the United States in the last few decades. The
2 most important factors in determining a childs outlook are the stage and histology of the
tumor. (The histology refers to how the cancer cells look under the microscope see What
is Wilms tumor?) Some of these rates are based on only small numbers of cases, so they
might not be accurate.
Survival rates are based on previous outcomes of children who had the disease, but they
cannot predict what will happen in any particular childs case. Knowing the stage and
histology of a childs Wilms tumor are important in estimating their outlook. But other
factors may also affect a childs outlook, such as how well the tumor responds to treatment.
Even when taking other factors into account, survival rates are only rough estimates. Your
childs doctor can tell you if the numbers below apply, as he or she is familiar with your
childs situation.
The treatment information in this document is not official policy of the Society and is not intended as medical
advice to replace the expertise and judgment of your cancer care team. It is intended to help you and your
family make informed decisions, together with your doctor.
Your doctor may have reasons for suggesting a treatment plan different from these general treatment options.
Dont hesitate to ask him or her questions about your treatment options.
A pediatric surgeon or pediatric urologist (doctor who treats urinary system problems in
children [and genital problems in boys])
A pediatric oncologist (doctor who uses chemotherapy and other medicines to treat
childhood cancers)
A pediatric radiation oncologist (doctor who uses radiation therapy to treat cancer in
children)
Many other specialists may be involved in your childs care as well, including physician
assistants, nurse practitioners, nurses, psychologists, social workers, rehabilitation specialists,
and other health professionals. For more information, see our document Children Diagnosed
With Cancer. Understanding the Health Care System.
After your childs tumor is found and its stage and histology are determined, the cancer care
team will discuss treatment options with you. Its important to discuss all of the options as
well as their possible side effects with your childs doctors so you can make an informed
decision. (For a list of some questions to ask, see the section What should you ask your
childs doctor about Wilms tumor?)
If time permits, it can often be helpful to get a second opinion if you have questions about the
recommended plan (or if you just want to confirm that its the best option). This can provide
you with more information and help you feel more confident about the treatment plan you
choose.
The main types of treatment that can be used for Wilms tumor are:
Surgery
Chemotherapy
Radiation therapy
Most children will get more than one type of treatment.
In the United States, surgery is the first treatment for most Wilms tumors. In Europe, doctors
prefer to give a short course of chemotherapy before the surgery. There seems to be no
difference in the results from these 2 approaches.
The first goal of treatment is to remove the primary (main) tumor even if the cancer has
spread to distant parts of the body. Sometimes the tumor may be hard to remove. It may be
very large, it may have spread into nearby blood vessels or other vital structures, or it may be
in both kidneys. For these children, doctors might use chemotherapy, radiation therapy, or a
combination of the 2 to try to shrink the tumor(s) before surgery.
If any cancer cells remain after surgery, radiation therapy or more surgery may be needed.
The next few sections describe the types of treatments used for Wilms tumors. This is
followed by a description of the most common approaches used for these tumors based on
the tumor stage (extent) and histology (appearance under a microscope).
Please see the Additional resources for Wilms tumor section for other, more specific
materials on the different types of cancer treatments and their side effects.
removes the cancer along with the whole kidney, the fatty tissue around the kidney, the ureter
(tube that carries urine from the kidney to the bladder), and the attached adrenal gland (a
hormone-making gland that sits on top of the kidney). Most children do very well with
only one kidney.
Partial nephrectomy (nephron-sparing surgery): In the small number of children who
have Wilms tumors in both kidneys, the surgeon will try to save some normal kidney tissue,
if possible. The surgeon may remove the kidney containing the most tumor with a radical
nephrectomy. In the other kidney the surgeon may do a partial nephrectomy, removing just
the tumor and a margin of normal kidney around it. Another option may be to do partial
nephrectomies on both kidneys.
Sometimes, both kidneys may need to be removed completely. The child will then need
dialysis several times a week. In this procedure, a machine does the job of the kidneys by
filtering waste products out of the blood. Once the child is healthy enough, a kidney
transplant may be an option if a donor kidney becomes available.
called a venous access device, central venous catheter, or just aport. The tube might be put
in during the main surgery to remove the tumor, or as a separate operation (especially if
chemo is going to be given before the main surgery).
The end of the tube will be just under the skin or sticking out of the chest area or upper arm.
This can be left in place for several months to take blood samples for tests and to give
intravenous (IV) chemotherapy drugs and blood transfusions, without having to use a needle
in the arm each time. Members of the cancer care team will teach you how to care for your
childs venous access device to reduce the risk of problems, such as clotting and infections.
Hair loss
Mouth sores
Loss of appetite
Nausea and vomiting
Diarrhea or constipation
Increased chance of infections (from having too few white blood cells)
Easy bruising or bleeding (from having too few blood platelets)
Fatigue or extreme tiredness (from having too few red blood cells)
Your childs doctor and treating team will watch closely for any side effects that develop.
There are often ways to lessen these side effects. For example, drugs can be given to help
prevent or reduce nausea and vomiting. Be sure to ask your childs doctor or nurse about
medicines to help reduce side effects, and let him or her know if your child has side effects
so they can be managed.
Along with the effects listed above, some drugs can have specific side effects. For example:
Vincristine can damage nerves. Some patients may have tingling, numbness, weakness,
or pain, particularly in the hands and feet.
Doxorubicin may cause heart damage. The risk of this happening goes up as the total
amount of the drug given goes up. Doctors try to limit this risk as much as possible by
not giving more than the recommended doses and by checking the heart with a test called
an echocardiogram (an ultrasound of the heart) during treatment.
Cyclophosphamide can damage the bladder, which can cause blood in the urine. The risk
of this can be lowered by giving the drug with plenty of fluids and with a drug called
mesna, which helps protect the bladder.
Lab tests to check for chemo side effects
Before each chemotherapy session, your childs doctor will check blood test results to see
how well the liver, kidneys, and bone marrow are working. If there are problems, the chemo
might need to be delayed or the doses reduced. You can learn more about these tests and
what they mean in Understanding Your Lab Test Results.
Long-term side effects of chemo
Possible long-term effects of treatment are one of the major challenges children might face
after cancer treatment.
For example, if your child is given doxorubicin (Adriamycin), there is a chance it could
damage the heart. Your childs doctor will carefully watch the doses used and will check
your childs heart function with imaging tests.
Some chemo drugs can increase the risk of developing a second type of cancer (such as
leukemia) years after the Wilms tumor is cured. But this small increase in risk has to be
weighed against the importance of chemotherapy in treating Wilms tumor. (See Second
Cancers Caused by Cancer Treatment to find out more about this.)
Some drugs might also affect fertility (the ability to have children) years later.
See the section What happens after treatment for Wilms tumor? for more on the possible
long-term effects of treatment.
For more information on chemotherapy in general, see the Chemotherapy section of our
website, or our document Understanding Chemotherapy: A Guide for Patients and Families.
Effects on skin areas that receive radiation can range from mild sunburn-like changes and
hair loss to more severe skin reactions.
Radiation that reaches the chest area can affect the heart and lungs. This does not usually
cause problems right away, but in some children it might lead to heart or lung problems
as they get older.
In girls, radiation may damage the ovaries. This might lead to abnormal menstrual cycles
or problems getting pregnant or having children later on.
Radiation slightly increases the risk of developing a second cancer in the area, usually
many years after it is given. This doesnt happen often with Wilms tumor because the
amount of radiation used is low.
See the section What happens after treatment for Wilms tumor? for more on the possible
long-term effects of treatment.
For more detailed information on radiation therapy, see the Radiation section of our
website, or our document Understanding Radiation Therapy: A Guide for Patients and
Families.
Look for red flags that suggest fraud. Does the method promise to cure all or most
cancers? Are you told not to use regular medical treatments? Is the treatment a secret
that requires you to take your child to certain providers or to another country?
Talk to your childs doctor or nurse about any method you are thinking about.
Contact us at 1-800-227-2345 to learn more about complementary and alternative
methods in general and to find out about the specific methods you are looking at. You
can also read about them in the Complementary and Alternative Medicine section of
our website.
Stage II
These tumors have grown outside the kidney into nearby tissues, but surgery removed all
visible signs of cancer.
Favorable histology: After surgery, standard treatment is chemo with actinomycin D and
vincristine. If the tumor cells have certain chromosome changes, the drug doxorubicin
(Adriamycin) may be given as well. The chemo is given for several months.
Unfavorable histology with focal (only a little) anaplasia: When the child recovers from
surgery, radiation therapy is given over several weeks. When this is finished, chemo
(doxorubicin, actinomycin D, and vincristine) is given for about 6 months.
Unfavorable histology with diffuse (widespread) anaplasia: After surgery, these children
get radiation over several weeks. This is followed by a more intense type of chemo using the
drugs vincristine, doxorubicin, etoposide, cyclophosphamide, and carboplatin, along with
mesna (a drug that protects the bladder from the effects of cyclophosphamide), which is
given for about 6 months.
Stage III
These tumors were not removed completely with surgery because of their size or location or
for other reasons. In some cases, surgery to remove the tumor may be postponed until other
treatments are able to shrink the tumor first (see below).
Favorable histology: Treatment is usually surgery if it can be done, followed by radiation
therapy over several days. This is followed by chemo with 3 drugs (actinomycin D,
vincristine, and doxorubicin). If the tumor cells have certain chromosome changes, the drugs
cyclophosphamide and etoposide may be given as well. Chemo is given for about 6 months.
Unfavorable histology with focal (only a little) anaplasia: Treatment starts with surgery if
it can be done, followed by radiation therapy over several weeks. This is followed by chemo,
usually with 3 drugs (actinomycin D, vincristine, and doxorubicin) for about 6 months.
Unfavorable histology with diffuse (widespread) anaplasia: Treatment starts with surgery
if it can be done, followed by radiation therapy over several weeks. This is followed by
chemo, usually with the drugs vincristine, doxorubicin, etoposide, cyclophosphamide, and
carboplatin, along with mesna (a drug that protects the bladder from the effects of
cyclophosphamide). Chemo lasts about 6 months.
In some instances the tumor may be very large or may have grown into nearby blood vessels
or other structures so that it cannot be removed safely. In these children, a small biopsy
sample is taken from the tumor to be sure that its a Wilms tumor and to determine its
histology. Then chemo is started. Usually the tumor will shrink enough within several weeks
so that surgery can be done. If not, then radiation therapy might be given as well. Chemo will
be started again after surgery. If radiation was not given before surgery, its given after
surgery.
Stage IV
These tumors have already spread to distant parts of the body at the time of diagnosis. As
with stage III tumors, surgery to remove the tumor might be the first treatment, but it might
need to be delayed until other treatments can shrink the tumor (see below).
Favorable histology: Surgery to remove the tumor is the first treatment if it can be done,
followed by radiation therapy. The entire abdomen will be treated if there is still some cancer
left after surgery. If the cancer has spread to the lungs, low doses of radiation might also be
given to that area. This is followed by chemo, usually with 3 drugs (actinomycin D,
vincristine, and doxorubicin) for about 6 months. If the tumor cells have certain chromosome
changes, the drugs cyclophosphamide and etoposide may be given as well.
Unfavorable histology: Treatment might start with surgery if it can be done, followed by
radiation therapy. The entire abdomen will be treated if there is still some cancer left after
surgery. Low doses of radiation will also be given to both lungs if there is spread to the
lungs. This is followed by chemo with the drugs vincristine, doxorubicin, etoposide,
cyclophosphamide, and carboplatin, along with mesna given for about 6 months. If the tumor
cells have diffuse (widespread) anaplasia, some doctors might try the chemo drugs irinotecan
and vincristine first instead (although this is not yet a commonly used treatment). The
treatment would then be adjusted based on if the tumor shrinks in response to these drugs.
If the tumor is too large or has grown too much to be treated safely with surgery first, a small
biopsy sample may be taken from the tumor to be sure that its a Wilms tumor and to
determine its histology. Chemo and/or radiation therapy may then be used to shrink the tumor.
Surgery may be an option at this point. This would be followed by more chemo and radiation
therapy if it wasnt given already.
For stage IV cancers that have spread to the liver, surgery may be an option to remove any
liver tumors that still remain after chemo and radiation therapy.
Stage V
Treatment for children with tumors in both kidneys is unique for each child, although it
typically includes surgery, chemo, and radiation therapy at some point.
Biopsies (tissue samples) of tumors in both kidneys and of nearby lymph nodes may be taken
first, although not all doctors feel this is needed because when both kidneys have tumors, the
chance that they are Wilms tumors is very high.
Chemo is typically given first to try to shrink the tumors. The drugs used will depend on the
extent and histology (if known) of the tumors. After about 6 weeks of chemo, surgery (partial
nephrectomy) may be done to remove the tumors if enough normal kidney tissue can be left
behind. If the tumors havent shrunk enough, treatment may include more chemo or radiation
therapy for about another 6 weeks. Surgery (either partial or radical nephrectomy) may then be
done. This is followed by more chemo, possibly along with radiation therapy if it hasnt been
given already.
If not enough functioning kidney tissue is left after surgery, a child may need to get dialysis, a
procedure where a special machine filters waste products out of the blood several times a
week. If there is no evidence of any cancer after a year or two, a donor kidney transplant may
be done.
Favorable histology
Initial stage of I or II
Emotional and social issues for Wilms tumor survivors and their
families
Most children with Wilms tumors are very young at the time of diagnosis. Still, some children
may have emotional or psychological issues that need to be addressed during and after
treatment. Depending on their age, they may also have some problems with normal
functioning and school work. These can often be overcome with support and encouragement.
Doctors and other members of the health care team can also often recommend special support
programs and services to help children after treatment.
Parents and other family members can also be affected, both emotionally and in other ways.
Some common family concerns include financial stresses, traveling to and staying near the
cancer center, the possible loss of a job, and the need for home schooling.
Centers that treat many patients with Wilms tumors may have programs to introduce new
patients and their families to others who have finished their treatment. This can give parents
an idea of what to expect during and after treatment, which is very important. Seeing another
patient with Wilms tumor doing well is often helpful.
Support groups for families of children with cancer can also be helpful. If you need help
finding such a group, call your American Cancer Society at 1-800-227-2345 and we can put
you in touch with a group or resource that may work for you.
If your child stayed in the hospital, a copy of the discharge summaries doctors prepare
when patients are sent home.
If chemotherapy was given, a list of the final doses of each drug your child received.
(Certain drugs may have specific long-term side effects. If you can get a list of these from
the pediatric oncologist, it might help any new doctors your child has in the future.)
If radiation therapy was given, a summary of the type and dose of radiation and when and
where it was given.
Its also very important to keep your health insurance. Follow-up tests and doctor visits cost a
lot, and even though no one wants to think of the tumor coming back, this could happen.
Books
The following books are available from the American Cancer Society. Call us at 1-800-227-
2345 or visit our bookstore online at cancer.org/bookstore to find out about costs or to place
an order.
For family and friends of the child
American Cancer Society Complete Guide to Family Caregiving, Second Edition
Because... Someone ILove Has Cancer (kids activity book)
Jacob Has Cancer: His Friends Want to Help (coloring book for a child with a friend who
has cancer)
For the child with cancer
Imagine Whats Possible: Use the Power of Your Mind to Take Control of Your Life During
Cancer (grades 4 through 6)
Let My Colors Out (picture book for ages S to 10)
The Long and the Short of It: A Tale About Hair (ages 7 and up)
National organizations and websites*
Along with the American Cancer Society, other sources of patient information and support
include:
American Childhood Cancer Organization (formerly Candlelighters)
Toll-free number: 1-855-858-2226
Website: www.acco.org
Offers information for children and teens with cancer, their siblings, and adults
dealing with children with cancer. Also offers books and a special kit for children
newly diagnosed with cancer, as well as some local support groups.
Childrens Oncology Group (COG)
Website: www.childrensoncologygroup.org
Provides key information from the worlds largest organization devoted to childhood
cancer research to help support children and their families from the time of diagnosis,
through treatment, and beyond. Also has a searchable database to find the COG
center closest to you.
CureSearch for Childrens Cancer
Toll-free number: 1-800-458-6223
Website: www.curesearch.org
Provides up-to-date information about childhood cancer from pediatric cancer
experts. Has sections on the website for patients, families, and friends to help guide
them on how to support the child with cancer.
National Cancer Institute
Toll-free number: 1-800-4-CANCER (1-800-422-6237)
TTY: 1-800-332-8615
Website: www.cancer.gov
Provides accurate, up-to-date information about cancer for patients and their families,
including clinical trials information. Offers a special booklet for teen siblings of a
child with cancer at: www.cancer.gov/cancertopics/when-your-sibling-has-cancer.
National Childrens Cancer Society, Inc.
Toll-free number: 1-800-5-FAMILY (1-800-532-6459)
Website: www.children-cancer.org
Services include an online support network for parents of children with cancer,
educational materials, and financial assistance for treatment-related expenses.
National Dissemination Center for Children with Disabilities (NICHCY)
Toll-free number: 1-800-695-0285 (also for TTY)
Website: www.nichcy.org
Provides information about disabilities and disability-related issues for families,
educators, and other professionals.
treatment schedules. Has online support groups, chat rooms, information, and more.
SuperSibs!
Toll-free number: 1-888-417-4704
Website: www.supersibs.org
Supports, honors, and recognizes 4- to 18-year-old brothers and sisters of children
diagnosed with cancer so they may face the future with strength, courage, and hope.
*Inclusion on this list does not imply endorsement by the American Cancer Society.
No matter who you are, we can help. Contact us anytime, day or night, for information and
support. Call us at 1-800-227-2345 or visit www.cancer.org.