|55 Kenosia Avenue
Danbury, CT 06810
Toll Free: 1.800.999.6673
The National Organization for Rare Disorders (NORD) web site, its databases, and the contents thereof are copyrighted by NORD. No part of the NORD web site, databases, or the contents may be copied in any way, including but not limited to the following: electronically downloading, storing in a retrieval system, or redistributing for any commercial purposes without the express written permission of NORD. Permission is hereby granted to print one hard copy of the information on an individual disease for your personal use, provided that such content is in no way modified, and the credit for the source (NORD) and NORD’s copyright notice are included on the printed copy. Any other electronic reproduction or other printed versions is strictly prohibited.
The information in NORD’s Rare Disease Database is for educational purposes only. It should never be used for diagnostic or treatment purposes. If you have questions regarding a medical condition, always seek the advice of your physician or other qualified health professional. NORD’s reports provide a brief overview of rare diseases. For more specific information, we encourage you to contact your personal physician or the agencies listed as “Resources” on this report.
Copyright 1990, 1991, 1992, 1994, 1995, 1996, 1997, 1998, 2003, 2005
Renal cell carcinoma is a form of kidney cancer. Some patients with renal cell carcinoma do not have symptoms (asymptomatic). When symptoms are present, they may include blood in the urine; urine that is brown or rusty-colored; abdominal pain; weight loss; enlargement of one testicle or varicose veins of the testis (varicocele) in a male patient; fever; a thin, malnourished appearance; vision abnormalities; and elevated blood pressure. The most common feature of the syndrome is the passing of blood in the urine (hematuria).
Renal cell carcinoma, though rare, is the most common form of kidney cancer found in adults. Usually the first sign that something is wrong is the passing of blood in the urine. Other signs may include flank pain and an abdominal mass that can be felt by the examining doctor. Other symptoms of renal cell carcinoma may include high blood pressure (hypertension), anemia, abnormal liver function and fever. Sometimes symptoms do not appear until the cancer has spread to another part of the body, usually the lymph nodes, lungs or the long bones.
The most common method of diagnosis is through the use of CT scans or sonography. It is very important to diagnose the disorder in the early stages so that prompt treatment can begin. Staging is a very important system to determine if and where the cancer has spread. Staging progresses from 1 to 4:
Stage 1 occurs when the tumor is confined to the kidney tissues themselves; Stage 2 occurs when the tumor involves the fat or adrenal tissues of the kidney; Stage 3 occurs when there is a tumor in the veins or vena cava of the kidney, the tumor has spread to the regional kidney nodes, or the tumor has involved lymph nodes and kidney veins or vena cava; Stage 4 occurs when the tumor has spread to other organs (liver, colon, pancreas, or stomach) or spread to distant sites in the body.
The exact cause of renal cell carcinoma is not known. However, a history of smoking does increase the risk for developing this disease. Patients with von Hippel-Lindau disease, horseshoe kidneys, adult polycystic kidney disease and kidney failure are also more prone to develop renal cell carcinoma.
Recent research suggests that two genes on the short arm of chromosome 3 (i.e., PRC and TFE 3) may be involved in the development of this particular type of malignancy. This form of kidney cancer has developed in several members of the same family, leading scientists to believe that there may be a genetic form of the disorder or perhaps a genetic predisposition toward developing renal cell carcinoma. However, exactly how the disease may be inherited is still unknown. Another gene, known as VHL, has also been linked with kidney cancer.
Renal cell carcinoma spreads (metastasizes) easily to the lungs and other organs.
Renal cell carcinoma is more common in males than in females (ratio of 2 or 3 to 1) and in persons with a history of smoking. It is also more common in persons with other types of kidney disorders and tends to run in some families. Renal cell carcinoma accounts for approximately 30,000 new cases of kidney malignancies per year in the United States.
Symptoms of the following disorders can be similar to those of renal cell carcinoma. Comparisons may be useful for a differential diagnosis:
Benign familial hematuria is a nonprogressive kidney disorder that usually begins in childhood and is characterized by red blood cells in the urine, and thinning of the microscopic parts of the kidney. It is often preceded by a respiratory infection. (For more information on this disorder, choose "Hematuria" as your search term in the Rare Disease Database.)
IGA nephropathy is a kidney disorder usually occurring during childhood and young adulthood. It usually follows a viral infection of the upper respiratory or gastrointestinal tracts. The major symptom is the passing of blood in the urine. There may be associated pain in the loin area. (For more information on this disorder, choose "IGA" as your search term in the Rare Disease Database.)
Polycystic kidney disease is an inherited disorder that is characterized by many cysts in both kidneys. This causes enlargement of the kidney size, while reducing the functional kidney tissue. In addition there may be pain in the loin area, blood in the urine, infection and colic. (For more information on this disorder, choose "Polycystic Kidney" as your search term in the Rare Disease Database.)
Additionally, many types of common kidney and bladder infections can cause blood to appear in the urine. These infections are treated with antibiotics.
Imaging studies, typically computerized tomography (CT) and abdominal ultrasonography (USG), are used in the diagnosis of renal cell carcinoma. Blood and urine testing may also be helpful.
The drug sorafenib (Nexavar) was approved in 2005 by the Food and Drug Administration (FDA) for the treatment of advanced renal cell carcinoma. Nexavar was developed through a partnership of Onyx Pharmaceuticals and the Bayer Pharmaceutical Corporation.
Treatment for kidney cancer often involves the surgical removal of all or part of the kidney (nephrectomy). This may also involve removal of the bladder or surrounding tissues. Hormone treatments may reduce the growth of the tumor in some cases. Chemotherapy may also be used.
Proleukin (interleukin-2 [IL-2]) was approved by the FDA in 1992 for the treatment of renal cell carcinoma. The drug is an anti-tumor agent usually given after surgery (nephrectomy) to slow tumor growth at sites to which the cancer may have spread (metastatic).
In 2009, Afinitor® (everolimus) tablets were approved by the US Food and Drug Administration (FDA) for patients with advanced renal cell carcinoma (RCC) after failure of treatment with Sutent® (sunitinib) or Nexavar® (sorafenib).
For more information contact:
One Health Plaza
East Hanover, NJ 07936-1080
Inlyta (also known as axitinib) by Pfizer Inc was approved by the FDA in 2012 as a treatment for patients with renal cell carcinoma who have not responded to another drug for this type of cancer.
Information on current clinical trials is posted on the Internet at www.clinicaltrials.gov. All studies receiving U.S. government funding, and some supported by private industry, are posted on this government website.
For information about clinical trials being conducted at the NIH Clinical Center in Bethesda, MD, contact the NIH Patient Recruitment Office:
Tollfree: (800- 411-1222
TTY: (866) 411-1010
For information about clinical trials sponsored by private sources, contact:
Information on cancer clinical trials is available through the Internet on www.cancer.gov or by calling: 1-800-4CANCER.
250 Williams NW St
Atlanta, GA 30303 USA
Phone #: 404-320-3333
800 #: 800-227-2345
Home page: http://www.cancer.org
American Society of Clinical Oncology
2318 Mill Road Suite 800
Alexandria, VA 22314
Phone #: 571-483-1780
800 #: 888-651-3038
Home page: http://www.cancer.net/
407 St John Street
London, EC1V 4AD United Kingdom
Phone #: 020- 72-42 0200
800 #: N/A
Home page: http://www.cancerresearchuk.org/cancer-help/
1050 17th St NW Suite 500
Washington, DC 20036
Phone #: 202-659-9709
800 #: 888-793-9355
Home page: http://www.cancersupportcommunity.org/
1800 M Street NW
Suite 1050 South
Washington, DC 22202
Phone #: 202-944-6700
800 #: N/A
Home page: http://www.focr.org
PO Box 8126
Gaithersburg, MD 20898-8126
Phone #: 301-251-4925
800 #: 888-205-2311
Home page: http://rarediseases.info.nih.gov/GARD/
c/o VHL Family Alliance
2001 Beacon Street, Suite 208
Boston, MA 02135-7787
Phone #: 617-277-5667
800 #: 800-767-4845
Home page: http://www.hlrccinfo.org
P.O. Box 803338 #38269
Chicago, IL 60680-3338 USA
Phone #: 847-332-1051
800 #: 800-850-9132
Home page: http://www.curekidneycancer.org
2201 E. Sixth Street
Austin, TX 78702
Phone #: 512-236-8820
800 #: 877-236-8820
Home page: http://www.livestrong.org
6116 Executive Blvd Suite 300
Bethesda, MD 20892-8322 USA
Phone #: 301-435-3848
800 #: 800-422-6237
Home page: http://www.cancer.gov
30 East 33rd Street
New York, NY 10016
Phone #: 212-889-2210
800 #: 800-622-9010
Home page: http://www.kidney.org
3 Information Way
Bethesda, MD 20892-3580
Phone #: N/A
800 #: 800-891-5390
Home page: http://www.kidney.niddk.nih.gov/
3400 Spruce Street
Philadelphia, PA 19104-4283 USA
Phone #: 215-349-8895
800 #: --
Home page: http://www.oncolink.upenn.edu
1649 North Pacana Way
Green Valley, AZ 85614 USA
Phone #: N/A
800 #: --
Home page: http://www.rare-cancer.org
1000 Corporate Blvd
Linthicum, MD 21090 USA
Phone #: 410-689-3700
800 #: 800-828-7866
Home page: http://www.urologyhealth.org/
Cecil Textbook of Medicine, 18th Ed.: James B. Wyngaarden, and Lloyd H. Smith, Jr., Editors; W.B. Saunders Co., 1988. Pp. 652-653.
Small Renal Neoplasms: Clinical Pathologic and Imaging Features. Levine, E.; et al.; AJR Am J Roentgenol (July, 1989, issue 153 (1) ). Pp. 69- 73.
Renal Cell Carcinoma. A Clinicopathologic and DNA Flow Cytometric Analysis of 103 Cases. McLemore, D. et al.; Cancer (November 15, 1989, issue 64 (10)). Pp. 2133-2140.
Therapeutic Options in Renal Cell Carcinoma. Buzaid, A.C. et al.; Semin Oncol, (February, 1989, issue 16 (1)). Pp. 12-19.
Role of Interferons in the Therapy of Metastatic Cell Carcinoma, Quesada, JR, Urology, (October, 1989, issue 34 (4) ). Pp. 80-83, discussion Pp. 87-96.
Renal-Cell Carcinoma. R. J. Motzer; New End J Med (Sep 19, 1996; 335(12)). Pp. 865-75.
Report last updated: 2012/02/06 00:00:00 GMT+0