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.
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 In. 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
Email: [email protected]
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.
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.
The information in NORD’s Rare Disease Database is for educational purposes only and is not intended to replace the advice of a physician or other qualified medical professional.
The content of the website and databases of the National Organization for Rare Disorders (NORD) is copyrighted and may not be reproduced, copied, downloaded or disseminated, in any way, for any commercial or public purpose, without prior written authorization and approval from NORD. Individuals may print one hard copy of an individual disease for personal use, provided that content is unmodified and includes NORD’s copyright.
National Organization for Rare Disorders (NORD)
55 Kenosia Ave., Danbury CT 06810 • (203)744-0100