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Pseudomyxoma Peritonei

Article
Digestive Health
Diseases & Conditions
+1
Contributed byKrish Tangella MD, MBAJan 03, 2019

What are the other Names for this Condition? (Also known as/Synonyms)

  • Gelatinous Ascites
  • Jelly Belly
  • Malignant Peritoneal Pseudomyxoma Peritonei

What is Pseudomyxoma Peritonei? (Definition/Background Information)

  • Pseudomyxoma Peritonei is the accumulation of abundant mucin (a type of gel-like protein) within the abdominal cavity, which is usually derived from a tumor in the appendix
  • However, rarely, this may be due to other tumors from the gastrointestinal tract, pancreas, or the ovary. Sometimes, the cause may not be due to a tumor at all
  • Treatment for the condition is dependent on the information, whether it was or was not caused by a malignancy, and on how much of the abdomen region is involved. Treatments may vary, from surgery, to a combination of chemotherapy and surgery, or chemotherapy alone
  • The prognosis of Pseudomyxoma Peritonei is hard to predict, and varies according to the cause of the condition (low-grade malignancy versus high-grade malignancy, or non-malignant tumors)

Who gets Pseudomyxoma Peritonei? (Age and Sex Distribution)

  • Pseudomyxoma Peritonei is more common in women
  • There is no age or ethnic predilection for this condition

What are the Risk Factors for Pseudomyxoma Peritonei? (Predisposing Factors)

There are no known risk factors associated with Pseudomyxoma Peritonei.

It is important to note that having a risk factor does not mean that one will get the condition. A risk factor increases ones chances of getting a condition compared to an individual without the risk factors. Some risk factors are more important than others.

Also, not having a risk factor does not mean that an individual will not get the condition. It is always important to discuss the effect of risk factors with your healthcare provider.

What are the Causes of Pseudomyxoma Peritonei? (Etiology)

  • Usually, Pseudomyxoma Peritonei is due to a tumor within the appendix that produces large amounts of mucin
  • When such a tumor has extended outside the appendix (rupture), it continues to produce gelatinous mucin, which accumulates within the abdominal cavity. The aggregates (elements) of mucin are usually accompanied by fragments of the tumor that have spilled into the abdominal cavity
  • This process may involve parts of, or the entire abdominal cavity, leading to the development of Pseudomyxoma Peritonei; a most severe clinical scenario
  • However, some cases are due to ovarian or other tumors that may also produce mucin, and progress in a similar manner to abdominal involvement. The behavior of ovarian and other rarer tumors are less understood, than appendix-derived tumors

What are the Signs and Symptoms of Pseudomyxoma Peritonei?

Signs and symptoms of Pseudomyxoma Peritonei include:

  • Increased abdominal girth and diffuse abdominal pain are the most common presenting signs and symptoms. Sometimes, the presenting sign is an inguinal hernia
  • Pseudomyxoma Peritonei may be asymptomatic in the early phase of the disease, and can be encountered unexpectedly, in 1 in 10,000 abdominal surgeries

How is Pseudomyxoma Peritonei Diagnosed?

A diagnosis of Pseudomyxoma Peritonei is made using a combination of:

  • Physical examination findings
  • Imaging studies (CT scan)
  • If necessary, sampling of the abdominal contents by needle (needle biopsy), or an open laparoscopic tissue biopsy, may be used

Many clinical conditions may have similar signs and symptoms. Your healthcare provider may perform additional tests to rule out other clinical conditions to arrive at a definitive diagnosis.

What are the possible Complications of Pseudomyxoma Peritonei?

Pseudomyxoma Peritonei can lead to the following complications:

  • Inflammation of the abdominal cavity (peritonitis)
  • Sepsis (infection of the blood)
  • Bowel obstruction

Death has been known to occur with advanced stages of Pseudomyxoma Peritonei.

How is Pseudomyxoma Peritonei Treated?

  • Treatment for Pseudomyxoma Peritonei is dependent on whether or not it is caused by a malignancy, and how much of the abdomen is involved
  • The treatments may vary from surgery, combination of chemotherapy and surgery, or chemotherapy alone
  • This is a controversial area amongst physicians and the treatment strategies may vary between different healthcare institutions

How can Pseudomyxoma Peritonei be Prevented?

Currently, there are no methods to prevent Pseudomyxoma Peritonei occurrence.

What is the Prognosis of Pseudomyxoma Peritonei? (Outcomes/Resolutions)

  • The prognosis of Pseudomyxoma Peritonei is hard to predict, and varies according to the cause of the condition; whether the tumor is of low-grade malignancy, or high-grade malignancy, or is non-malignant
  • However, it generally has a poor prognosis with 10-year survival rates varying from 0-70%. This broad range can be explained by the fact that this disease may be caused by aggressive cancers, and by non-cancerous causes, such as a ruptured bowel

Additional and Relevant Useful Information for Pseudomyxoma Peritonei:

The following DoveMed website link is a useful resource for additional information:

https://www.dovemed.com/healthy-living/digestive-center/

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Krish Tangella MD, MBA picture
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Krish Tangella MD, MBA

Pathology, Medical Editorial Board, DoveMed Team

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