For moms who have children that have or had a Wilm's Tumor. Wilm's tumor is one of the most common Kidney Cancers and about 400 children in the US get diagnosed with this every year. By the time a parent finds this tumor it had already grown to a size of 1-2 lbs. There is a theory that the cause for this cancer is due to immature kidney cells that may start in in utero. These cells start growing rapidly. Please read below information from Boston's Childrens Hospital.
What is Wilms' tumor (WT)?
Wilms' tumor, also called nephroblastoma, is a malignant (cancerous) tumor originating in the cells of the kidney. It is the most common type of renal (kidney) cancer and accounts for about 6 percent of all childhood cancers. Approximately 400 children in the United States are diagnosed with a Wilms' tumor each year. The disease can occur at any age between infancy and 15 years, but, in most cases, the tumor is detected by the age of 3. This cancer affects males and females equally.
The tumor can be very large and it may spread (metastasize) to other body tissues. The most common site for Wilm's tumor to metastasize is the lungs. Lesions may also occur, however, in the liver, the other kidney, brain, and/or bones. In approximately 5 percent of children with Wilms' tumor, both kidneys are involved.
As you read further below, you will find general information about Wilms' tumor. If you would like to view summary information about cancer first, see the cancer overview.
What causes Wilms' tumor?
It is uncommon for Wilms' tumor to run in families. Less than 2 percent of cases will have an affected relative. Most cases of Wilms' are considered sporadic (occur by chance) and are the result of genetic mutations that affect cell growth in the kidney. These mutations generally arise after birth, but, in some cases, children are born with a genetic alteration that predisposes them to cancer.
A small percentage of patients with Wilms' tumor have one of three genetic syndromes, including the following:
WAGR syndrome - the acronym WAGR stands for the four diseases present in WAGR syndrome, including: Wilms' tumor, aniridia (absence of the iris, the colored part of the eye), genitourinary malformations, and mental retardation. This is caused by loss or inactivation of a tumor suppressor gene called WT1 on chromosome #11. Tumor suppressor genes usually suppress the growth of tumors and control cell growth. When altered, they no longer control cell growth, and tumors may form.
Denys-Drash syndrome - this syndrome is characterized by kidney failure, genitourinary malformations, and tumors of the gonads (ovaries or testes). This is also caused by loss or inactivation of a tumor suppressor gene called WT1 on chromosome #11. Tumor suppressor genes usually suppress the growth of tumors and control cell growth. When altered, they no longer control cell growth, and tumors may form.
Beckwith-Wiedemann syndrome - this syndrome is characterized by large birthweight and a large liver, spleen, and tongue; low blood sugar in neonatal period, malformations around the ear, asymmetric growth of the body, abdominal wall defects near the navel (omphalocele), and tumors of the liver and adrenal glands. This is thought to be caused by an overactive copy of an oncogene on chromosome #11, called IGF2. Oncogenes control cell growth, but, if mutated, uncontrolled cell growth may result.
What are the symptoms of Wilms' tumor?
The following are the most common symptoms of a Wilms' tumor. However, each child may experience symptoms differently. Symptoms may include:
a non-tender mass, or lump, felt or seen in the abdomen
swelling of the abdomen
veins that appear distended or large across the abdomen
blood in the urine (hematuria)
pain in the abdomen from pressure on other organs near the tumor
decreased appetite and weakness or tiredness
high blood pressure (hypertension)
If a tumor is suspected in the child's abdomen, it is important not to apply pressure to this area. Careful bathing and handling of the child is necessary before and during any tumor evaluation. Rupture of the tumor may lead to cancer cells spreading to other tissues in the body. These symptoms of Wilms' tumor may resemble other medical conditions or serious illnesses. Always consult your child's physician for a diagnosis.
How is Wilms' tumor diagnosed?
In addition to a complete medical history and physical examination, diagnostic procedures may include, but are not limited to, the following:
abdominal ultrasound - a diagnostic imaging technique which uses high- frequency sound waves and a computer to create images of blood vessels, tissues and organs; can provide an outline of the kidneys, the tumor, and determine if there are problems in the renal or other major veins in the abdomen. It can also determine if there are any lesions or tumors in the opposite kidney.
abdominal computerized tomography scan (also called a CT or CAT scan) - a diagnostic imaging procedure that uses a combination of x-rays and computer technology to produce cross-sectional images (often called slices), both horizontally and vertically, of the body. A CT scan shows detailed images of any part of the body, including the bones, muscles, fat, and organs. CT scans are more detailed than x-rays. This is particularly useful for detecting any tumor that has spread to the lungs.
magnetic resonance imaging (MRI) - a diagnostic procedure that uses a combination of large magnets, radiofrequencies, and a computer to produce detailed images of organs and structures within the body. MRI can determine if there are metastases (spreading), if there are any tumor cells in the lymph nodes, and/or if any other organs are involved. Wilm's tumors can compress other organs in the area causing effects on their function. This technique is most sensitive to determine whether a clot of the tumor extends from the kidney vein through the vena cava towards the heart.
chest x-ray - a diagnostic test which uses invisible electromagnetic energy beams to produce images of internal tissues, bones, and organs onto film. A chest x-ray can determine if there are metastases (spreading) in the lungs.
blood and urine tests - to evaluate kidney and liver function.
biopsy - a sample of tissue that is removed and examined under a microscope. A biopsy of the tumor helps evaluate cells, extent of disease, and diagnosis.
surgical removal of the tumor and kidney (nephrectomy) - surgery may be necessary for a definitive diagnosis and determining the extent of the disease.
What are the treatments for Wilms' tumor?
Specific treatment for Wilms' tumor will be determined by your child's physician based on:
your child's age, overall health, and medical history
the extent of the disease
your child's tolerance for specific medications, procedures, or therapies
how your child's physician expects the disease may progress
your opinion or preference
Major advancements have occurred in treatment of this tumor over the last two decades, and this progress is the result primarily of studies performed by the National Wilms' Tumor Study Group of which Children's Hospital Boston is a member. This study group has completed four studies in which the chemotherapy and/or radiotherapy treatment has been randomized for children based on the stage of the tumor. These studies have defined the most effective treatments and, in the last two studies, have actually shown that briefer courses of therapy are adequate to obtain equivalent cure to longer courses of treatment. In this tumor, even children who have spread of the tumor to their lungs, can have a very high survival rate with appropriate treatment.
Surgical removal of the tumor continues to play a major role in the treatment of Wilms' tumor. The type of chemotherapy treatment and whether or not radiation is required are determined by the stage (which is the extent of spread of the tumor) as well as the subtype of the tumor (which is determined by the pathologist when he or she examines the tumor under the microscope).
Initial operative removal is the first step in the treatment and cure of these children. Only in certain instances where there is either a very massive tumor present at the time the tumor is first identified, or extensive presence of tumor within the lungs or extension of the tumor into the major veins draining the kidney is chemotherapy administered before resection of the tumor.
A summary of these treatments follows:
surgery - to remove all or part of the affected kidney and any involved structures, performed by a surgeon
biopsy - needed if the tumor is too large or involved in surrounding structures to be removed; a biopsy is also necessary for diagnosis and staging the disease, and will be done if a suspicious area is found when the opposite kidney is examined during the initial operation.
chemotherapy - a drug treatment that works by interfering with the cancer cell's ability to grow or reproduce. Different groups of drugs work in different ways to fight cancer cells and shrink tumors. Chemotherapy may be used alone for some types of cancer or in conjunction with other therapy such as radiation or surgery. Often, a combination of chemotherapy drugs is used to fight a specific cancer. Certain chemotherapy drugs may be given in a specific order depending on the type of cancer it is being used to treat. While chemotherapy can be quite effective in treating certain cancers, the agents do not differentiate normal healthy cells from cancer cells. Because of this, there can be many adverse side effects during treatment. Being able to anticipate these side effects can help the care team, parents, and child prepare, and, in some cases, prevent these symptoms from occurring, if possible.
Chemotherapy is systemic treatment, meaning it is introduced to the bloodstream and travels throughout the body to kill cancer cells. Chemotherapy can be given:
as a pill to swallow
as an injection into the muscle or fat tissue
intravenously (directly to the bloodstream; also called IV)
intrathecally - chemotherapy given directly into the spinal column with a needle
radiation therapy - using high-energy rays (radiation) from a specialized machine to damage or kill cancer cells and shrink tumors
stem cell transplant - a treatment involving stem cells, a specific type of cell from which all blood cells develop. Stem cells develop into red blood cells to carry oxygen, white blood cells to fight disease and infection, and platelets to aid in blood clotting. Transplantation of normal stem cells from another person is used to help restore normal blood production in patients whose own ability to make any or all of these blood cells has been compromised by cancer, intensive cancer treatment, or other types of damage or abnormality. The use of cells from another individual is called allogeneic transplantation. Stem cells collected from patients themselves prior to intensive treatment can also be used to supplement the recovery of the patient's own cells after particularly aggressive course of chemotherapy or radiation therapy. The use of a patient's own cells is referred to as autologous transplantation.
Stem cell transplantation and the treatment needed to manage its effects are complex. Your physician will give you more detailed information on what to expect.
blood pressure monitoring - essential when a kidney tumor is present
supportive care - any type of treatment to prevent and treat infections, side effects of treatments, and complications, and to keep your child comfortable during treatment
continuous follow-up care - a schedule of follow-up care determined by your child's physician and other members of your care team to monitor ongoing response to treatment and possible late effects of treatment, and to evaluate function of the remaining kidney
What is the long-term outlook for patients with Wilms' tumor?
The factors for determining the prognosis and long-term survival of children with Wilms' tumor include the following:
histology, favorable or unfavorable
extent of the disease
age and overall health of the child at diagnosis
size of the primary tumor
response to therapy
your child's tolerance of specific medications, procedures, or therapies
new developments in treatment
As with any cancer, prognosis and long-term survival can vary greatly from child to child. Prompt medical attention and aggressive therapy are important for the best possible prognosis. Continued follow-up care is essential for the child diagnosed with a Wilms' tumor. Side effects of chemotherapy and radiation, as well as second malignancies, can occur in survivors of cancer. New methods are continually being discovered to improve treatment and to decrease its long-term associated complications