Pseudomyxoma peritonei is a condition that occurs when tumor cells produce an abundance of mucin and this mucin pools in the abdominal cavity. Mucin is a protein that forms gels in the body. The tumors that usually cause this condition originate in the appendix.
Pseudomyxoma peritonei is a rare condition diagnosed in one to two people per million people each year. The occurrence of pseudomyxoma peritonei is slightly higher in women than it is in men with a 9:11 ratio. Unlike other cancers that spread by the blood stream or the lymphatic system, pseudomyxoma peritonei generally do not travel outside of the abdomen.
It can, however, affect the organs and systems surrounding it. As the mucin pools in the abdominal cavity it can begin to compress the colon, liver, and stomach. The cause of pseudomyxoma peritonei is unknown.
Signs & Symptoms
People who suffer from pseudomyxoma peritonei can experience symptoms such as abdominal pain, bloating and a loss of appetite. They may experience digestive disorders, a change in their bowl habits or changes in their weight. In some cases pseudomyxoma peritonei can cause appendicitis or infertility.
Diagnosis
Because pseudomyxoma peritonei is so rare it is often diagnosed during surgery for a different, unrelated condition. CT scans and tumor markers are helpful to monitor the cancer once it is discovered.
Treatment
Treatment for pseudomyxoma peritonei varies based on the severity of the mucin build up and the problems this may or may not be causing. Pseudomyxoma peritonei is very slow growing so “watchful waiting” is the best treatment for some patients. Other may require surgery.
Doctors may remove some or all of the mucin in what is known as debulking. In some cases they also remove the surrounding organs that may have been affected. This can include parts of a woman’s reproductive organs such as the ovaries and fallopian tubes. In both men and women the gall bladder, spleen, and parts of the intestines and stomach, may be removed.
Prognosis
Unfortunately prognosis for patients with pseudomyxoma peritonei is fairly poor. There is an extremely high recurrence rate since it is often discovered late and it is difficult to remove all of the affected cells. Treatment can help alleviate symptoms, but there is currently no cure.
