$Unique_ID{BRK03562} $Pretitle{} $Title{Carcinoma, Renal Cell} $Subject{Carcinoma Renal Cell RCC Kidney Cancer Renal Adenocarcinoma Grawitz Tumor Hypernephroma Nephrocarcinoma Hematuria Benign Familial IGA Nephropathy Polycystic Kidney Disease} $Volume{} $Log{} Copyright (C) 1990, 1991, 1992 National Organization for Rare Disorders, Inc. 815: Carcinoma, Renal Cell ** IMPORTANT ** It is possible that the main title of the article (Renal Cell Carcinoma) is not the name you expected. Please check the SYNONYM listing to find the alternate names and disorder subdivisions covered by this article. Synonyms RCC Kidney Cancer Renal Adenocarcinoma Grawitz Tumor Hypernephroma Nephrocarcinoma Information on the following diseases can be found in the Related Disorders section of this report: Hematuria, Benign Familial IGA Nephropathy Polycystic Kidney Disease 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. Renal Cell Carcinoma is a rare malignant kidney disorder. Major symptoms may include loss of kidney function, fever, weight loss, blood problems, high levels of calcium in the system, high blood pressure, blood clots and congestive heart failure. However, the most common feature of the syndrome is the passing of blood in the urine (hematuria). Symptoms 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. Causes The exact cause of Renal Cell Carcinoma is not known. Some scientists believe that the disorder is more common in persons who smoke pipes and/or cigars. 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 the short arm of chromosome 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 at least a genetic predisposition toward developing Renal Cell Carcinoma. However, just how the disease is inherited is still unknown. Affected Population Renal Cell Carcinoma is more common in males than in females (ratio of 2 or 3 to 1) and in persons who smoke pipes or cigars. 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 6% or approximately 18,000 new cases of kidney malignancies per year in the United States. Related Disorders 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. Therapies: Standard Treatment of Renal Cell Carcinoma depends on which of the four stages the tumor is in at the time of diagnoses. Surgery is the treatment of choice for tumors that are primary and have not spread to other parts of the body. CT scans, MRI scans, scans of the veins (venacavogram), bone scans and chest X- rays are very important tools in judging whether or not the tumor has spread beyond the kidney. When spreading has occurred treatment may include radiotherapy (radiation), chemotherapy, immunotherapy, and hormonal therapy. The drug Vinblastine is often used to treat Renal Cell Carcinoma. Proleukin (interleukin-2) was approved by the FDA in May of 1992 for the treatment of Renal Cell Carcinoma. The drug is manufactured by Chiron Corp. and is an anti-tumor agent usually given after surgery (nephrectomy) to slow tumor growth at sites to which the cancer may have spread (metastatic). Proleukin (interleukin-2, IL-2) is manufactured by the Cetus Corporation and also is an anti-tumor agent usually given after surgery (nephrectomy) to slow tumor growth at sites to which the cancer may have spread (metastatic). Therapies: Investigational These orphan drugs are being tested with the end in views of treatings sites to which cancer may have spread. Roferon-A (interferon alfa-2a2,6) and Intron A (interferon alfa-2b2,6). The orphan drug Poly I: C12u (Ampligen) is being developed by HEM Research Inc., 1617 JFK Blvd., Suite 600, Philadelphia, PA, 19103, as a treatment for Renal Cell Carcinoma. Further testing is necessary to determine the long-term safety and effectiveness of these and other drugs in the treatment of Renal Cell Carcinoma. The FDA has given approval for testing of the orphan drug r-IFN-beta (Interferon Beta (Recombinant)) for treatment of systemic Metastatic Renal Cell Carcinoma. The drug is manufactured by Biogen, Inc., 14 Cambridge Center, Cambridge, MA, 02142. This disease entry is based upon medical information available through June 1992. 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 Renal Cell Carcinoma, please contact: National Organization for Rare Disorders P.O. Box 8923 New Fairfield, CT 06812-1783 (203) 746-6518 American Cancer Society 1599 Clifton Rd., NE Atlanta, GA 30329 (404) 320-3333 The National Kidney Foundation 30 East 33rd St. New York, NY 10016 212-689-2210 or 1-800-622-9010 NIH/National Kidney and Urologic Diseases Information Clearinghouse Box NKUDIC Bethesda, MD 20892 301-468-6345 NIH/National Cancer Institute (NCI) 9000 Rockville Pike, Bldg. 31, RM. 1A24 Bethesda, MD 20892 1-800-4-CANCER References 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, AC, et al. Semin Oncol, (February, 1989, issue 16 (1)). Pp. 12-19. ROLE OF INTERFERONS IN THE THERAPY OF METASTATIC RENAL CELL CARCINOMA, Quesada, JR, Urology, (October, 1989, issue 34 (4) ). Pp. 80-83, discussion Pp. 87-96.