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$Unique_ID{BRK04147}
$Pretitle{}
$Title{Pseudomyxoma Peritonei}
$Subject{Pseudomyxoma Peritonei Malignant Appendiceal Tumor Malignant Large
Cystadenocarcinoma Malignant Large Peritoneal Carcinomatosis Malignant Large
Bowel Tumor Appendicitis Carcinoid Syndrome Adenocarcinoma of the Appendix}
$Volume{}
$Log{}
Copyright (C) 1991 National Organization for Rare Disorders, Inc.
843:
Pseudomyxoma Peritonei
** IMPORTANT **
It is possible that the main title of the article (Pseudomyxoma
Peritonei) is not the name you expected. Please check the SYNONYM listing to
find the alternate names and disorder subdivisions covered by this article.
Synonyms
Malignant Appendiceal Tumor
Malignant Large Cystadenocarcinoma
Malignant Large Peritoneal Carcinomatosis
Malignant Large Bowel Tumor
Information on the following diseases can be found in the Related
Disorders section of this report:
Appendicitis
Carcinoid Syndrome
Adenocarcinoma of the Appendix
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.
Pseudomyxoma Peritonei is a rare malignant growth lining the abdominal
cavity. It usually develops from a cancer located in the appendix, goblet
cells of the large bowel, or ovary. It may fill up the abdominal area
causing swelling of the abdomen and generalized pain in the lower abdomen.
It often takes years to develop.
Symptoms
Pseudomyxoma Peritonei is usually characterized by pain in the lower abdomen
which can suggest an appendicitis attack. Upon examination the abdomen is
found to be swollen but not painful to the touch. Surgery to remove the
appendix reveals the abdominal cavity to be filled with tumors of mucus
origin (mucinous) and mucinous swelling of the abdomen (ascites). In many
cases the abdomen is completely filled with these mucinous tumors. However,
the small bowel is usually spared allowing for removal of much of the
tumorous growth. Serious consequences of the disorder may be loss of
intestinal function and intestinal obstruction.
Causes
Pseudomyxoma Peritonei is generally thought to develop very slowly from the
spread of appendix, large bowel or ovarian cancer to the abdominal area, as
the spreading of a mucinous intra-abdominal tumor progresses, it begins to
fill up the abdomen with an "Omental cake" resulting in a condition known as
"jelly-belly".
Affected Population
Pseudomyxoma Peritonei is a very rare disease. Only about 450 cases are
mentioned in the medical literature. It affects people in a ration of one
man to every four women. It is more common in women because it often
develops from an ovarian tumor. It primarily occurs in older persons, the
mean age being 57 years of age.
Related Disorders
Symptoms of the following disorders can be similar to those of Pseudomyxoma
Peritonei. Comparisons may be useful for a differential diagnosis:
Appendicitis is a common inflammation of the appendix, a small organ
located near the first part of the large intestine. The inflammation results
from a bacterial infection that causes the appendix to swell and fill with
pus. Symptoms include intermittent pain in the navel region. In time the
pain becomes more severe and localized in the lower, right quadrant of the
abdomen. The lower abdomen becomes tender and touching increases the pain
dramatically.
Carcinoid Syndrome is a rare, malignant disease of slow growing tumors
affecting the small bowel, stomach and/or pancreas. The syndrome may be
accompanied by stomach pain, blockage of arteries and hot flushes. (For more
information on this disorder, choose "Carcinoid Syndrome" as your search term
in the Rare Disease Database).
Adenocarcinoma of the Appendix is the most common form of malignancy
found in the appendix. It is often difficult to distinguish Adenocarcinoma
of the Appendix from non-cancerous mucoceles. Both types may cause
appendicitis.
Therapies: Standard
The use of CAT and Ultrasound scans can often identify Pseudomyxoma Peritonei
before surgery. Imaging can show if mucinous cells have produced large
amounts of mucus in the abdominal cavity.
Surgery along with intraperitoneal chemotherapy can often increase
disease free life expectancy if the cancer has not spread from another
location. Drugs used in treating Pseudomyxoma Peritonei include Mitomycin C
and 5-Flourouracil.
Therapies: Investigational
Studies are ongoing in surgical and medicinal ways of treating all forms of
cancer including Pseudomyxoma Peritonei. For more information on this
research contact:
Dr. Paul H. Sugarbaker
The Cancer Institute
Washington Hospital Center
110 Irving Street, NW
Washington, DC 20010-1975
Resources
For more information on Pseudomyxoma Peritonei, please contact:
National Organization for Rare Disorders (NORD)
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
NIH/National Cancer Institute (NCI)
9000 Rockville Pike, Bldg. 31, Rm 1A2A
Bethesda, MD 20892
(800) 4-CANCER
The National Cancer Institute has developed PDQ (Physician Data Query), a
computerized database designed to give the public, cancer patients and
families, and health professionals quick and easy access to many types of
information vital to patients with this and many other types of cancer. To
gain access to this service, call:
Cancer Information Service (CIS)
1-800-4-CANCER
In Washington, DC and suburbs in Maryland and Virginia, 636-5700
In Alaska, 1-800-638-6070
In Oahu, Hawaii, 1-808-524-1234 (Neighbor islands call collect)
References
CURRENT THERAPY IN COLON AND RECTAL SURGERY, "Cancer of the Appendix and
Pseudomyxoma" Paul H. Sugarbaker, B.C. Decker, 1990.
MALIGNANT PSEUDOMYXOMA PERITONEI OF COLONIC ORIGIN. NATURAL HISTORY AND
PRESENTATION OF A CURATIVE APPROACH TO TREATMENT., PH Sugarbaker, et al.; Dis
Colon Rectum, (October, 1987, issue 30 (10)). Pp. 772-779.
PSEUDOMYXOMA PERITONEI PRESENTING AS A SCROTAL MASS., WC Baker, et al.; J
Urol (April, 1988, ISSUE 139 (4)). Pp. 821-822.
A 25-YEAR REVIEW OF ADENOCARCINOMA OF THE APPENDIX. A FREQUENTLY
PERFORATING CARCINOMA., MA Cerame, Dis Colon Rectum, (February, 1988, issue
31 (2)). Pp. 145-150.