$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.