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- $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.
-
-