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Diseases

Genetic and Rare Diseases Information Center (GARD)

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Pseudomyxoma peritonei


Other Names for this Disease
  • PMP
  • Syndrome of pseudomyxoma peritonei
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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 is a tumor that most often begins in the appendix.  As the tumor grows, tumor cells may spread into the peritoneal cavity and land on other nearby organs (such as the ovaries or intestines), sometimes forming additional tumors on the surface of these organs.[1]  Pseudomyxoma peritonei is extremely unlikely to grow into these organs (to become invasive) or spread to more distant parts of the body (metastasize); therefore, it is not usually thought of as a malignant tumor.[1]  It is rare, occurring in approximately two out of a million people, and women are affected two to three times more frequently than men.[1]  The first symptoms of pseudomyxoma peritonei are an increase in the size of the belly, appendicitis, or the finding of a mass on the ovary during medical imaging tests.[2]  
Last updated: 9/27/2011

How might pseudomyxoma peritonei be treated?

Pseudomyxoma peritonei is first treated with surgery to remove as much of the tumor as possible (debulking).[2][1][3]  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,[1] 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.[2][3]  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.[2][3] 
Last updated: 9/25/2011

References
  • 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.
  • 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.
  • 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.