Pseudomyxoma Peritonei (PMP Cancer)- Symptoms, Causes & Treatment

Pseudomyxoma Peritonei Syndrome: An Overview

Pseudomyxoma Peritonei (PMP), also called jelly-belly, literally means "false mucinous tumor of the peritoneum." It is a type of cancer so rare that it only affects 2 in a million individuals.

PMP occurs when mucin-producing cancer cells spread inside the peritoneum (the smooth tissue lining of the abdominal organs). The symptoms of this disease tend to be mild, like bloating or indigestion. The unusual signs make its detection very difficult.

But there is hope for the people who are diagnosed with PMP. Keep reading to learn the causes, symptoms, diagnosis, and treatment options for Pseudomyxoma peritonei.

What is Pseudomyxoma Peritonei Cancer?

Pseudomyxoma Peritonei Cancer is a very rare case of cancer that impacts your peritoneal cavity (your abdomen and pelvis). It fills your abdomen and pelvis with cancer cells that generate mucin. Mucin is a jelly-like substance that’s one of the  mucus component.

The name of this medical condition means ‘false mucinous tumor of the peritoneum’. Doctors call this disease a 'false tumour' primarily because its initial growth is dominated by the accumulation of large amounts of jelly-like mucin (mucinous ascites) rather than a solid, single mass. However, the cancer cells themselves do form neoplastic tissue, known as peritoneal implants, which proliferate and spread throughout the abdomen. In contrast, the disease initiates as a cancerous polyp in your appendix.

When the polyp  breaks by your appendix. It triggers a flood of mucin-producing  cancer cells that, with the time, impact your digestive system. That abundance of jelly-like mucin is why some  people known PMP “Jelly belly”.

Symptoms of pseudomyxoma peritonei tend to be mild and may feel like common issues such as constipation or indigestion. In some cases, doctor identifies PMP early on while performing your yearly medical examination or while checking for another medical condition. In case you are like most people, however, you will find out you have PMP because your belly issues do not go away or get worse.

What causes Pseudomyxoma Peritonei (PMP)?

The Psemyxoma Peritonia begins in the appendix. A small perforation forms in the appendix due to a polyp or tumor formed on its surface. The polyp ruptures, bursting the appendix along with it. Mucin-producing cancer cells flood the abdominal and pelvic cavities.

Wherever the cells reach, they continue to grow. The most affected areas are the fatty membranes on the intestines, below the muscle that separates the chest from the abdomen, and the pelvis. In women, there can be an excessive growth of cancerous cells in both ovaries.

The mucin is a jelly-like substance, hence the name Jelly-Belly. As is the case of most cancers, no one knows why the tumors form.

What are the signs and symptoms of PMP?

Pseudomyxoma peritonei is not an aggressive cancer. Early PMP is typically asymptomatic. Advanced cases may cause progressive abdominal distension (jelly belly), bowel obstruction, or ovarian involvement in women.. The typical signs include:

• Noticeable abdominal swelling or bloating or Abdominal distension.
• Abdominal Painor a feeling of continued discomfort in the abdomen caused by the "jelly-like" mucin build-up.
• Blockage of the large intestine, causing constipation.
• Hernias or new lumps in the abdomen.
• Loss of appetite and feeling full on eating too little.
• Difficulty in getting pregnant due to pressure from mucin buildup or inflammation in your reproductive organs can make you difficult to conceive in case of women.
• Nausea

None of these signs are atypical. These are subtle symptoms that get overlooked as mild gastric issues and PMP detection get delayed.

What is the diagnosis of Pseudomyxoma Peritonei?

A person is diagnosed with PMP when their symptoms have become severe. The doctor orders the following tests:

• Blood test: Complete blood count and blood tests for markers that might indicate cancerous signs.
• Imaging: Ultrasound of the whole abdomen, a CT scan, or an MRI.
• Diagnostic laparoscopy: If the imaging shows tumors, a biopsy of the mucinous fluid or tumors is performed.
   

What are the treatment options for Pseudomyxoma Peritonei?

The chief treatment for PMP is surgery. A peritonectomy (a cytoreductive surgery) will help remove the mucin and the mucinous cysts throughout the abdomen. The doctors prescribe either chemotherapy, radiation, or both after surgery.

Pseudomyxoma peritonei can recur even after surgery. But with care and proper treatment, people go on to live long, fulfilling lives.

While there is no way to prevent PMP, paying attention to any abdominal symptoms will help in early detection. Having a history of ovarian cancer puts those women at a greater risk.

What are the complications or side effects of surgery?

There is always an ultimate chance that surgery to remove organs will result into complications such as hemorrhage, infection or anastomotic leak. Around 1–2% of people die after the definitive PMP treatment, which is highly specialized Cytoreductive Surgery (CRS) combined with Hyperthermic Intraperitoneal Chemotherapy (HIPEC). This rate is an estimate for this complex, high-risk procedure performed in specialized centers, where major operative complications can occur in 20–30% of patients.

Treatments have been successful in 65% of patients, achieving a complete cure. With constant monitoring, early diagnosis, and family support, a healthy and improved life is possible.

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