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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.
-
-