Pseudomyxoma Peritonei

Pseudomyxoma Peritonei

National Organization for Rare Disorders, Inc.

Important

It is possible that the main title of the report Pseudomyxoma Peritonei is not the name you expected. Please check the synonyms listing to find the alternate name(s) and disorder subdivision(s) covered by this report.

Synonyms

  • colloid carcinoma
  • disseminated peritoneal adenomucinosis (DPAM)
  • malignant appendiceal tumor
  • malignant large bowel cystadenocarcinoma
  • malignant large bowel peritoneal carcinomatosis
  • malignant large bowel tumor
  • mucinous cyst adenocarcinoma
  • mucinous cystadenoma
  • peritoneal carcinomatosis
  • peritoneal mucinous carcinomatosis (PMCA)
  • pseudomyxoma ovarii
  • PMP

Disorder Subdivisions

  • None

General Discussion

Pseudomyxoma peritonei is a rare malignant growth characterized by the progressive accumulation of mucus-secreting (mucinous) tumor cells within the abdomen and pelvis. The disorder develops after a small growth (polyp) located within the appendix bursts through the wall of the appendix, and spreads mucus-producing tumor cells throughout the surrounding surfaces (e.g., the membrane that lines the abdominal cavity [peritoneum]). As mucinous tumor cells accumulate, the abdominal area becomes swollen and digestive (gastrointestinal) function becomes impaired. Pseudomyxoma peritonei develops at a variable rate, but may grow at a slower rate (indolent) than other malignancies within the abdomen.

Symptoms

The most common symptoms in individuals with pseudomyxoma peritonei occur due to progressively increasing mucinous tumor within the abdomen and pelvis. Usually, the most common symptom is increasing abdominal size (so called "jelly belly") and abdominal pain. Although the abdomen may be swollen, it is generally not painful to touch (palpation). In affected males, the second most common finding is protrusion of parts of the intestines through an abnormal opening in the muscular wall of the abdomen near the groin (inguinal hernia). In affected females, the second most common finding is usually an abnormally enlarged ovary. The mucinous tumor seems to grow rapidly within ovarian tissue.



The mucinous tumor accumulates within the fatty membrane in front of the intestines (greater omentum), beneath the muscle that separates the chest from the abdomen (diaphragm), and within the pelvis. In most cases, the small bowel is unaffected. Frequently, the primary tumor that punctured the appendix may be small in comparison to the extensive mucinous tumor that develops within the abdomen and pelvis. Without treatment intervention, this disorder will result in obstruction of the intestines or the loss of intestinal function. Progressive mucinous tumor accumulation can result in poor food intake, malnutrition, and, eventually, life-threatening complications.

Causes

The exact cause of pseudomyxoma peritonei is not known. There are no genetic, familial, or environmental factors known to cause this disorder.



Pseudomyxoma peritonei develops from a hole (perforation) in the appendix that is caused by the penetration of a small growth (polyp) located within the appendix. The tumor cells from the appendix spread (migrate) within the abdominal and pelvic cavity to characteristic locations. Once they reach these locations, tumor cells continue to grow. Characteristic locations for tumor cell growth are within the fatty membrane in front of the intestines (greater omentum), beneath the muscle that separates the chest from the abdomen (diaphragm), and within the pelvis. In females, there may be excessive growth on both ovaries.

Affected Populations

Pseudomyxoma peritonei is a very rare disorder with approximately 500 cases discussed in the medical literature. Males and females are affected in equal numbers. It is a misconception that females develop this disorder more frequently than males. However, this misconception occurs because some ovarian tumors are frequently mistakenly identified as pseudomyxoma ovarii.



The average age of onset of pseudomyxoma peritonei is 48 years, which is considerably earlier than other malignancies that occur within the abdomen.

Standard Therapies

The diagnosis of pseudomyxoma peritonei may be confirmed by advanced imaging technologies such as abdominal CT scan or abdominal MRI (magnetic resonance imaging). These imaging tests may reveal the characteristic distribution of large amounts of mucus to particular locations within the abdomen and pelvis. They may also localize a primary tumor at the base of the cecum referred to as a mucocele.



The goal of the treatment of pseudomyxoma peritonei is cure. This is achieved in approximately 65% of patients. The treatments are cytoreductive surgery with peritonectomy in an attempt to remove all visible evidence of the disease from the abdomen and pelvis. Then, to prevent reimplantation of cancer cells, the abdomen is washed with a warm chemotherapy solution. This is commonly referred to hyperthermic intraperitoneal chemotherapy or HIPEC. Sometimes the surgeon must search carefully for the primary appendiceal tumor for it may be very small in comparison to the mucinous tumor and mucinous ascites that can accumulate in kilogram quantities within the abdomen and pelvis. The hyperthermic inrtraperitoneal drugs which are commonly used to treat this disease include mitomycin C and oxaliplatin. The chemotherapy is heated to 42 degress in the abdominal cavity to increase penetration of the drugs into the mucinous tumor and to increase the local cytotoxicity.

Investigational Therapies

Information on current clinical trials is posted on the Internet at www.clinicaltrials.gov. All studies receiving U.S. government funding, and some supported by private industry, are posted on this government web site.



For information about clinical trials being conducted at the NIH Clinical Center in Bethesda, MD, contact the NIH Patient Recruitment Office:



Tollfree: (800) 411-1222

TTY: (866) 411-1010

Email: prpl@cc.nih.gov



For information about clinical trials sponsored by private sources, contact:

www.centerwatch.com

References

TEXTBOOKS

Ceelen WP. Peritoneal Carcinomatosis. New York, NY: Springer; 2007.



Sugarbaker P. Cancer of the appendix and pseudomyxoma. In: Current Therapy in Colon and Rectal Surgery. 2nd ed. Philadelphia, PA: BC Decker, Inc; 2005:369-378.



Sugarbaker, P. Intraperitoneal chemotherapy and cytoreductive surgery. 2nd ed. Grand Rapids, MI: Ludann Press:1995.



JOURNAL ARTICLES

JO Yan TD, Black D, Savady R, Sugarbaker PH. A systematic review on the efficacy of cytoreductive surgery and perioperative intraperitoneal chemotherapy for pseudomyxoma peritonei. Ann Surg Oncol. 2007;14:484-92.



Sugarbaker PH. New standard of care for appendiceal epithelial malignancies and pseudomyxoma peritonei syndrome. Lancet Oncol. 2006;7:69-76.



Galani E, et al. Pseudomyxoma peritonei: the 'controversial' disease. Int J Gynecol Cancer. 2003;13:413-8.



Van Ruth S, et al. Pseudomyxoma peritonei: a review of 62 cases. Eur J Surg Oncol. 2003;29:682-88.



Jivan S, Bahal V. Pseudomyxoma peritonei. Postgrad Med J. 2002;78:170-72.



Jackson SL, et al. Gelatinous ascites: a cytohistologic study of pseudomyxoma peritonei in 67 patients. Mod Pathol. 2001;14:664-71.



Sugarbaker PH, et al. Pseudomyxoma peritonei syndrome. Adv Surg. 1996;30:233-79.



Gough DB, et al. Pseudomyxoma peritonei: long-term patient survival with an aggressive regional approach. Ann Surg. 1994;219:112-19.



Smith JW, et al. Pseudomyxoma peritonei of appendiceal origin. Cancer. 1992;70:386-401.

Resources

American Cancer Society, Inc.

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PMP Pals' Network

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Fax: (831)424-4545

Email: pmppals@yahoo.com

Internet: http://www.pmppals.org



Rare Cancer Alliance

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Genetic and Rare Diseases (GARD) Information Center

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Lance Armstrong Foundation

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CORE

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PMP Research Foundation, Inc.

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Independence, OH 44131

USA

Tel: (216)986-7010

Fax: (216)524-9111

Email: info@pmpcure.org

Internet: http://www.pmpcure.org



For a Complete Report

This is an abstract of a report from the National Organization for Rare Disorders, Inc.® (NORD). Cigna members can access the complete report by logging into myCigna.com. For non-Cigna members, a copy of the complete report can be obtained for a small fee by visiting the NORD website. The complete report contains additional information including symptoms, causes, affected population, related disorders, standard and investigational treatments (if available), and references from medical literature. For a full-text version of this topic, see http://www.rarediseases.org/search/rdblist.html.

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