Pseudomyxoma peritonei, or PMP, is considered a rare form of cancer that typically starts in your appendix (which is why it’s also called appendix cancer). It is estimated to affect about two people per million, and it’s unknown what causes this unusual cancer to occur. However, as more becomes known about this disease, doctors are discovering it is not as rare as once thought.
PMP usually begins with a polyp, or small growth, on the lining of your appendix (similar to the polyps that may occur with colon cancer). Less often, the cancer may start in your bowel, ovary or bladder.
The polyp typically causes no symptoms in the early stages but continues to grow until it bursts through your appendix wall and into your abdominal cavity.
At this point additional tumors may develop in your abdomen, which secrete a jelly-like fluid called mucin. Unlike most forms of cancer, which spread through your bloodstream or lymphatic system, PMP stays isolated primarily in your abdomen and spreads via mucin.
As mucin builds up in your abdomen, symptoms may begin to appear. These include:
- Pain in your abdomen or pelvic region
- Abdominal swelling and bloating
- Changes in bowel habits
- Loss of appetite
- Bowel irritation
- Fertility problems
Not everyone who has PMP will experience these symptoms. Further, because it is considered rare, PMP is often misdiagnosed. In women, PMP may be confused with acute appendicitis or ovarian cancer (which can also cause abdominal swelling and produce mucin).
PMP may also be confused with colon cancer or even irritable bowel syndrome, so a thorough examination is necessary. In many cases, a laparotomy (surgery to look into your abdomen) is required for proper diagnosis.
If you’re experiencing any of the symptoms listed above with an unknown cause, getting checked for appendix cancer is important, as the earlier it’s found the easier it is to treat. There are three classifications for PMP diagnosis:
- Low grade
- High grade, which means aggressive cancer cells called signet ring cells have also been found
- Hybrid, which falls between low grade and high grade
Treatment is often based on “the Sugarbaker technique,” which is named for its inventor Dr. Paul Sugarbaker. It involves cytoreductive surgery to first remove mucin and tumors form your abdominal cavity, followed by intraoperative hyperthermic peritoneal chemotherapy (HIPEC), which is liquid chemotherapy poured directly into your abdominal cavity following surgery.
You may also be given early postoperative intraperitoneal chemotherapy (EPIC) for several days after the surgery, which involves liquid chemotherapy administered to your abdomen via a port. The surgery and HIPEC treatment is an involved treatment that may take 10 to 14 hours or more to complete.
Finding a specialist who is familiar with PMP and its unique treatment options is essential to receiving the best outcomes. If you or a family member has been diagnosed with PMP, you can find further support online at the PMP Research Foundation which allows you to connect to and share information with others affected by PMP. Please take a few moments to check out the video below to learn more about PMP and Appendix Cancer.
PMP Research Foundation
KVUE January 17, 2016
Living Better with Cancer