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Diseases

Genetic and Rare Diseases Information Center (GARD)

Pseudomyxoma peritonei


Other Names for this Disease
  • PMP
  • Syndrome of pseudomyxoma peritonei
See Disclaimer regarding information on this site. Some links on this page may take you to organizations outside of the National Institutes of Health.

Your Question

I have been diagnosed with a benign pseudomyxoma peritonei.  Most of the information I have found relates to the malignant type.  I had a hysterectomy and appendectomy with debulking three weeks ago.  I was told I had a large ovarian tumor plus a tumor on my appendix, which seemed to be the one secreting mucus. What is the treatment for this condition?

Our Answer

We have identified the following information that we hope you find helpful. If you still have questions, please contact us.

What is pseudomyxoma peritonei?

Pseudomyxoma peritonei (PMP) is a rare condition characterized by the presence of mucin in the abdominal (peritoneal) cavity. While the most common cause of PMP is appendix cancer, several types of tumors (including non-cancerous tumors) can cause PMP.[1] Signs and symptoms may include an increase in abdominal size or bloating; inguinal hernia (in men); an ovarian mass, felt during a routine pelvic exam (in women); pain or discomfort in the abdomen; and/or appendicitis.[2][1] Treatment depends on the underlying cause of the condition (the location and type of the original tumor, including whether it is malignant) and the extent of spreading.[1] It may involve surgically removing as much of the tumor as possible (debulking); radiotherapy; and/or chemotherapy.[1][2]
Last updated: 4/11/2016

How might pseudomyxoma peritonei be treated?

Pseudomyxoma peritonei is first treated with surgery to remove as much of the tumor as possible (debulking).[3][4][5]  Additional surgeries may be needed to remove more of the tumor if it is not completely removed during the first surgery.  Chemotherapy is most often used to treat cancers that might spread to other parts of the body (metastasize).  Though pseudomyxoma peritonei is extremely unlikely to metastasize,[4] it has a high chance of regrowing after surgery if all tumor cells are not removed.  As such, chemotherapy may be placed directly into the peritoneal cavity during surgery for pseudomyxoma peritonei to destroy any tumor cells that might remain but are too small to be seen in order to prevent the tumor from regrowing.[3][5]  Though the use of chemotherapy during surgery has not been compared directly with surgery alone, chemotherapy during surgery is considered the standard treatment for pseudomyxoma peritonei.[3][5] 
Last updated: 9/25/2011

References
  • About ACPMP. ACPMP Research Foundation. http://acpmp.org/about-acpmp.
  • Richard Swanson, Jeffrey A Meyerhardt. Cancer of the appendix and pseudomyxoma peritonei. UpToDate. Waltham, MA: UpToDate; March, 2016;
  • Sugarbaker PH. New standard of care for appendiceal epithelial neoplasms and pseudomyxoma peritonei syndrome?. Lancet Oncology. 2006; 7:69-76. http://www.ncbi.nlm.nih.gov/pubmed/16389186. Accessed 9/23/2011.
  • Smeenk RM, Bruin SC, van Velthuysen ML, Verwaal VJ. Pseudomyxoma Peritonei. Current Problems in Surgery. 2008; 45:527-575. http://www.ncbi.nlm.nih.gov/pubmed/18590843. Accessed 9/21/2011.
  • Yan TD, Black D, Savady R, Sugarbaker PH. A systematic review on the efficacy of cytoreductive surgery and perioperative intraperitoneal chemotherapy for pseudomyxoma peritonei. Annals of Surgical Oncology. 2007; 14:484-492. http://www.ncbi.nlm.nih.gov/pubmed/17054002. Accessed 9/21/2011.
Other Names for this Disease
  • PMP
  • Syndrome of pseudomyxoma peritonei
See Disclaimer regarding information on this site. Some links on this page may take you to organizations outside of the National Institutes of Health.