Pseudomxyoma Peritonei

What is Pseudomyxoma Peritonei?

Pseudomyxoma Peritonei
is a mucin-producing tumor usually originating from the appendix.
This is a condition in which cells have spread from the appendix or ovaries into the abdominal or peritoneal cavity.

Pseudomyxoma Peritonei generally develops as a small polyp-like growth, or adenoma, originating with the appendix. Eventually the adenoma breaks or tears through the appendix, spreading mucin or gelatinous ascites, throughout the peritoneum.
The mucin is thick and viscous, often producing large tumors or clusters of tumors. These mucinous tumors  of gelatinous ascites, enlarge causing bloating and abdominal distension. If left unchecked, mucinous tumors will eventually cause bowel obstruction. The mucin must be surgically removed.
Due to the viscosity (density and gelatinous nature) of the mucin, it cannot be removed via paracentesis or liposuction.


Pseudomyxoma Peritonei generally remains within the peritoneum or abdominal cavity and rarely spreads via the blood or lymphatic systems.
Appendiceal carcinoid tumors represent approximately fifty per cent of all appendix tumor cases. Carcinoid tumors are usually less than two centimeters in size and rarely spread to lymph nodes.
Appendiceal non-carcinoid tumors originate from the epithelial cells lining the inside of the appendix. These cells create tumors producing mucin (a gelatinous material defined as Pseudomyxoma Peritonei.)  These tumor cells and mucin (Pseudomyxoma Peritonei) can accumulate (increase) gradually taking more space within the abdominal or peritoneal cavity. If these mucin producing cells spread outside the appendix and into the peritoneal or abdominal cavity, they can lead to fatal bowel obstruction, if left untreated.
Appendiceal adenocarcinoid tumors and goblet cell carcinomas are similar to both carcinoid and adenocarcinoma tumors of the appendix.The designation of the Pseudomyxoma Peritonei syndrome is divided into categories as follows:

 (1)
Mucinous Ascites:  acellular mucin within the peritoneum;


 (2) Organized mucinous fluid: mucin accumulation as a result of tissue granulation and inflammatory cells;
 

(3) Disseminated Peritoneal Adenomucinosis aka DPAM
: intraperitoneal mucin with epithelium showing low-grade atypia.  Accumulation of mucin outside the right lower quadrant of the abdomen may indicate a more challenging prognosis.
(4)  Mucin accompanied by abundant malignant epithelium, high-grade atypia (Ronnett et al, 1995) is described as Peritoneal Mucinous Carcinomatosis. Mucinous Carcinomatosis indicates a more challenging prognosis compared to the more common pattern of DPAM.
"Pseudomyxoma Peritonei" describes thick mucinous content in the peritoneal cavity, developing from  the  rupture of a mucocele from the appendix (more common) or from the rupture of a cystic neoplasm originating from an ovary (less common.)

4 comments:

  1. Do you have this condition? I am interested in how you got it. I am afraid a surgeon made a mistake during a removal of my mucocele of my appendix and that I may be at risk for this disease.
    Thank you

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  2. Yes I have this disease. It's been consistently reported to appear around the age of 45, which happened in my case. It starts as a cyst in the appendix or ovaries, which pushes through and ruptures which then spreads a mucinous gel throughout the peritoneal cavity.

    My suggestion would be to speak to your doctor about your concerns. This is considered an orphan disease, 1 in 1 million will be diagnosed with it. They still don't know exactly what causes it or who is at risk, so express your concerns and educate your self on the subject, knowledge is important when it comes to your health care.

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  3. I have done a lot of research, and I am curious as to what amount of mucin release it takes to cause the disease. My doctor admits to releasing mucus during the procedure. I am meeting with him this week.
    I have not found any research or articles that indicate that a small amount of mucin release can cause pmp.

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  4. Go to:http://www.pmppals.org/index.html
    You will find the most current and accurate information available on this matter.
    I wish I knew what to say about your situation, this must be hard to deal with, but I understand.
    I do recommend getting a copy of your pathology report if you have not already been given one after your surgery. Go over it with your doctor if something doesn't make sense, get answers.
    And as for the disease, if the mucin is diseased it spreads through the peritoneal cavity and adheres to organs and intestines, it's a slow growing disease that takes about 2-3 years to get to the point of needing surgery.
    Keep in touch and let me know how your appointment went, I'll keep you in my thoughts.

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