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$Unique_ID{BRK04332}
$Pretitle{}
$Title{Wilms' Tumor}
$Subject{Wilms' Tumor Nephroblastoma Embryoma Kidney Embryonal Carcinosarcoma
Kidney Embryonal Mixed Tumor Kidney Embryonal Adenomyosarcoma Kidney Renal
Cell Carcinoma Transitional Cell Carcinoma }
$Volume{}
$Log{}

Copyright (C) 1987, 1990 National Organization for Rare Disorders, Inc.

343:
Wilms' Tumor

** IMPORTANT **
It is possible the main title of the article (Wilms' Tumor) is not the
name you expected.  Please check the SYNONYMS listing to find the alternate
names, disorder subdivisions, and related disorders covered by this article.

Synonyms

     Nephroblastoma
     Embryoma Kidney
     Embryonal Carcinosarcoma Kidney
     Embryonal Mixed Tumor Kidney
     Embryonal Adenomyosarcoma Kidney

Information on the following disorders can be found in the Related
Disorders section of this report:

     Renal Cell Carcinoma
     Transitional Cell Carcinoma

General Discussion

** REMINDER **
The information contained in the Rare Disease Database is provided for
educational purposes only.  It should not be used for diagnostic or treatment
purposes.  If you wish to obtain more information about this disorder, please
contact your personal physician and/or the agencies listed in the "Resources"
section of this report.


Wilms' Tumor is the most common form of kidney cancer in children,
accounting for six to eight percent of all childhood cancers.  The exact
cause is not known, although it is thought to be inherited in some cases.  An
abdominal swelling is the most common symptom usually leading to early
detection of the disease.  Wilms' Tumor can often be treated successfully
depending on the stage of the tumor at detection and the age and general
health of the child.  Approximately eighty percent of children with this
disease can be cured with appropriate treatment.

Symptoms

In the early stages, Wilms' Tumor usually has no apparent symptoms.  Later
signs may include blood in the urine, low-grade fever, loss of appetite,
paleness, weight loss, lethargy, and a swelling of the abdomen.  In the later
stages, pain may be intermittent and slight, or sudden and sharp.  These
symptoms could be due to a variety of other disorders, but parents who
observe such symptoms should consult a doctor promptly.

Developmental anomalies may also be apparent in patients with Wilms'
Tumor.  These may include absence of the colored portion of the eye or iris
(Aniridia) which occurs in approximately ten percent of patients, an abnormal
enlargement of a part of the body (hemihypertrophy) which occurs in about
three percent, and various genitourinary defects in about five percent of
cases.

Causes

The exact causes of Wilms' Tumor are not known at this time.  Scientists
believe that there are both hereditary and nonhereditary forms of the
disease.

The hereditary type of Wilms' Tumor may arise at an earlier age and is
likely to affect both kidneys or several sites in one kidney.  Scientists
suspect that a genetic abnormality causes a defect in the fetal kidney, and
genetic studies have tentatively identified the location of this chromosome.
When the disorder is inherited it is usually transferred through a recessive
trait.  (Human traits including the classic genetic diseases, are the product
of the interaction of two genes for that condition, one received from the
father and one from the mother.  In recessive disorders, the condition does
not appear unless a person inherits the same defective gene from each parent.
If one receives one normal gene and one gene for the disease, the person will
be a carrier for the disease, but usually will show no symptoms.  The risk of
transmitting the disease to the children of a couple, both of whom are
carriers for a recessive disorder, is twenty-five percent.  Fifty percent of
their children will be carriers, but healthy as described above.  Twenty-five
percent of their children will receive both normal genes, one from each
parent and will be genetically normal.)

Affected Population

Wilms' Tumor accounts for six to eight percent of all childhood cancers.
The disorder affects one child in about 10,000 in the United States.  The
onset of Wilms' Tumor occurs mainly in children under the age of seven, with
most cases developing between the ages of one and four years of age.  Boys
and girls are affected equally.

Related Disorders

Renal Cell Carcinoma, the most common type of kidney cancer in adults,
arises in the lining of the kidney (renal) tubules.  Also known as renal
adenocarcinoma, hypernephroma, and Grawitz' Tumor, it accounts for more than
eighty-five percent of all adult kidney cancers.

Transitional Cell Carcinoma of the renal pelvis is the next most common
form of adult kidney cancer.  The symptoms of this disorder resemble bladder
cancer in many ways.

Therapies:  Standard

In general, treatment of Wilms' Tumor is usually successful.  Treatment
programs that combine modern surgical techniques (including kidney removal),
radiation therapy, and chemotherapy (Actinomycin D and vincristine can
reduce the size of Wilms' Tumors), have brought dramatic progress in
treating this disease.

Children treated for Wilms' Tumor are usually considered cured if they
survive for two years without any sign that the disease has returned.  The
small number of children with aggressive cell types, or with widespread
disease at the time of diagnosis have a poorer outlook, but many of these
patients are curable with intensive therapy.

Treatment centers have adopted a modern team approach in caring for
Wilms' Tumor patients.  With pediatric surgeons, radiation therapists and
doctors who specialize in treatment of tumors (oncologists) working together,
these centers have consistently reported overall two-year survival rates of
seventy to eighty percent in children treated for Wilms' Tumor.

Chemotherapy and radiation therapy can cause undesirable side effects
such as nausea and vomiting.  A more serious short-term side effect is the
suppression of bone marrow.  In killing cancer cells, chemotherapy and
radiation therapy can also destroy some normal cells in the bone marrow.  If
the white cells, red cells, and platelets fall too low, patients can become
susceptible to bleeding and infection.  Such side effects are usually
temporary.  Supportive care, such as antibiotic therapy, often helps to
protect patients from complications during their treatment.

Therapies:  Investigational

Researchers have reported that there may be a link between tumor growth in
the patients with Wilms' Tumor and a deletion of the p13 section of
chromosome 11.  Further research may indicate the site of the gene that
controls the cancer cell growth and enable the scientist to suppress the
cells' ability to form tumors.

The National Wilms' Tumor Study (NWTS), was conducted by a national
consortium of hospitals and clinics, with funding from the National Cancer
Institute for the purpose of carrying out controlled clinical trials of new
treatments for Wilms' Tumor.  Findings of this study are the basis for
treatment standards for Wilms' Tumor patients in the United States.
Researchers are now considering ways to improve the care of patients with
spreading cancer or those with cell types shown at high risk by previous
research.

This disease entry is based upon medical information available through
February 1990.  Since NORD's resources are limited, it is not possible to
keep every entry in the Rare Disease Database completely current and
accurate.  Please check with the agencies listed in the Resources section for
the most current information about this disorder.

Resources

For more information on Wilms Tumor, please contact:

     National Organization for Rare Disorders (NORD)
     P.O. Box 8923
     New Fairfield, CT  06812-1783
     [phone]

     The National Kidney Foundation
     2 Park Ave.
     New York, NY  10016
     [phone]
     [phone]

     American Kidney Fund
     6110 Executive Blvd., Suite 1010
     Rockville, MD  20852
     [phone]
     [phone]
     [phone] (MD)

     American Cancer Society
     1599 Clifton Rd., NE
     Atlanta, GA  30329
     [phone]

     NIH/National Cancer Institute
     9000 Rockville Pike, Bldg. 31, Rm. 1A2A
     Bethesda, MD 20892
     1-800-4-CANCER

The National Cancer Institute has developed PDQ (Physician Data Query), a
computerized database designed to give the public, cancer patients and
families, and health professionals quick and easy access to many types of
information vital to patients with this and many other types of cancer.  To
gain access to this service, call:

     Cancer Information Service (CIS)
     1-800-4-CANCER
     In Washington, DC and suburbs in Maryland and Virginia, 636-5700
     In Alaska, 1-800-638-6070
     In Oahu, Hawaii, [phone] (Neighbor islands call collect)

For genetic information and genetic counseling referrals, please contact:

     March of Dimes Birth Defects Foundation
     1275 Mamaroneck Avenue
     White Plains, NY  10605
     [phone]

     Alliance of Genetic Support Groups
     35 Wisconsin Circle, Suite 440
     Chevy Chase, MD  20815
     (800) 336-GENE
     [phone]

References

ADULT KIDNEY CANCER AND WILMS' TUMOR:  RESEARCH REPORT.  U.S. Department of
Health and Human Services, Public Health Service, National Cancer Institute,
National Institutes of Health.