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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.