$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 (203) 746-6518 The National Kidney Foundation 2 Park Ave. New York, NY 10016 (212) 889-2210 (800) 622-9010 American Kidney Fund 6110 Executive Blvd., Suite 1010 Rockville, MD 20852 (301) 881-3052 (800) 638-8299 (800) 492-8361 (MD) American Cancer Society 1599 Clifton Rd., NE Atlanta, GA 30329 (404) 320-3333 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, (808) 524-1234 (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 (914) 428-7100 Alliance of Genetic Support Groups 35 Wisconsin Circle, Suite 440 Chevy Chase, MD 20815 (800) 336-GENE (301) 652-5553 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.