Pseudomyxoma Peritonei (PMP)

Pseudomyxoma Peritonei (PMP) is a rare type of cancer characterized by a mix of tumor and mucin in the abdominal cavity. If left untreated, mucin will eventually build up to the point where it compresses vital structures: the colon, the liver, kidneys, etc. Unlike most cancers, PMP rarely spreads through the lymphatic system or through the bloodstream. Therefore PMP is characterized by mucin and scattered cancer cells in the abdominal cavity but not with liver or other sites of metastasis. PMP is most commonly associated with cancer of the appendix; mucinous tumors of the ovary have also been implicated. There have been efforts to rename PMP that begins in the appendix as low grade mucinous neoplasm of the appendix with disseminated peritoneal adenomucinosis.

PMP has different classifications based upon the malignancy of the tumor mass and the slow or aggressive growth pattern of the tumor and mucin itself.  These classifications are currently being standardized.  For the moment, PMP Is a group of diseases that may best be regarded as “peritoneal surface malignancies” (PSM), which include:

  • Appediceal (Appendix) cancer. [Audrey Hepburn was a notable victim of appendix cancer, dying of the disease in 1993. Yesterday would have been her 80th birthday.]
  • Dissemenated Peritoneal Adenomucinosis (DPAM) of any origin.
  • Peritoneal Mucinous Adenocarcinoma
  • Peritoneal Mucinous Carcinomatosis (PMCA) of any origin.

Caring for Cancer Patients – Support for Caregivers

People who take care of people with cancer need to remember to take care of themselves. Robert Bowden’s wife has been diagnosed with appendix cancer and is being treated at M. D. Anderson Cancer Center.

PMP was once thought to be diagnosed in about 1000 people world-wide each year.  It’s said that the lifetime odds of being diagnosed with PMP are about 1 in 1 million.  However, specialists now believe it is more common than was once thought.  For certain, PMP and PSM requires earlier & more accurate diagnostic tools. The average age of diagnosis for Pseudomyxoma Peritonei is approximately 44 years of age.

PMP Is listed among the rare or “orphan” diseases acknowledged by the National Association of Rare Disorders. An orphan disease is generally considered to have a prevalence of fewer than 200,000 affected individuals in the United States.  An orphan disease has such a low prevalence in the general population that a doctor in a busy general practice would not expect to see more than one case a year.  It also means that pathologists see the disease infrequently and may misinterpret cell pathology, resulting in an incorrect diagnosis. Clearly, improved awareness and diagnostic methods in the medical community will benefit patients through earlier detection and treatment of PMP/PSM.

PMP Research Foundation

Treatment for Pseudomyxoma Peritonei, and related diseases, generally includes surgery, with or without systemic or intraperitoneal heated chemotherapy (HIPEC). During cytoreductive surgery for Pseudomyxoma Peritonei, mucinous tumors are removed. The removal of these tumors may possibly involve the resection or removal of other organs or tissues to which the tumors have become attached. Due to several factors, radiation has not been an effective form of treatment for Pseudomyxoma Peritonei.

PMP Pals Network

Notable Tweet(s) and Upcoming Fundraiser:
@rare_cancer Spouse of pseudomyxoma peritonei, a cancer that originates in the appendix, survivor organizes first Bark for Life May 16, 2009 http://is.gd/whia
ds_nneigh_330
Darrell Sapp/Post-Gazette
Jodie Mohnkern has organized Bark for Life in Zelienople. Here she poses with holding Tucker, with Reuben, to the left, and Levi, in the forground.

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~ by marbleroad on May 5, 2009.

One Response to “Pseudomyxoma Peritonei (PMP)”

  1. Another great resource is Pseudomyxoma Survivor – http://www.pseudomyxomasurvivor.co.uk – and the Facebook support group for which you will find a link on the website

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