What is the definition of Pseudomyxoma Peritonei?
Pseudomyxoma peritonei (PMP) is a rare disease characterized by the presence of mucin in the abdominal (peritoneal) cavity. While the most common cause of PMP is appendix cancer, several types of tumors (including non-cancerous tumors) can cause PMP. Signs and symptoms may include an increase in abdominal size or bloating; inguinal hernia (in men); an ovarian mass that may be felt during a routine pelvic exam (in women); pain or discomfort in the abdomen; and/or appendicitis. Treatment depends on the underlying cause of the condition (the location and type of the original tumor, including whether it is malignant) and the extent of spreading. A combination of cytoreductive surgery and hyperthermic intraperitoneal chemotherapy is often the most successful treatment.
What are the alternative names for Pseudomyxoma Peritonei?
- Syndrome of pseudomyxoma peritonei
- Gelatinous ascites
What are the causes for Pseudomyxoma Peritonei?
The exact cause of pseudomyxoma peritonei (PMP) is currently unknown. No specific genetic or environmental factors have been found to cause PMP. Various types of tumors can lead to PMP, and it is not known why some cause PMP and others do not. Appendix tumors are the most common primary tumors associated with PMP, but tumors of the ovary, colon, stomach, pancreas, and urachus have also been reported in people with PMP.
It is generally thought that cells from a mucinous tumor (usually of the appendix) spread into the abdominal cavity, attaching themselves to the peritoneum and other organs in the abdomen. The tumor cells then continue to grow and produce mucin, which builds up and contributes to the signs and symptoms in people with PMP.
What are the current treatments for Pseudomyxoma Peritonei?
The treatment of pseudomyxoma peritonei depends on the underlying cause of the condition (the location and type of the original tumor, including whether it is malignant) and the extent of spreading. The approach depends on the size of the lesions and damage. It is needed to have an exploratory surgery (laparotomy) to find out which organs are affected. A combination of cytoreductive surgery and hyperthermic intraperitoneal chemotherapy is often the most successful treatment, and can achieve overall survival outcomes of up to 84% at 5 years. Cytoreductive surgery involves removing all lesions that can be removed. This is followed by hyperthermic intraperitoneal chemotherapy which involves placing heated chemotherapy drugs directly into the peritoneal cavity during surgery to destroy any remaining tumor cells that are too small to be seen.
Pressurized intraperitoneal aerosol chemotherapy is another option that can be combined with the surgery and have less complications of the hyperthermic form, but this treatment is still experimental and can be carried out only in specialized centers.The procedure is performed for 90 min under general anesthesia. The chemotherapy is vaporized in the form of an aerosol. Chemotherapy after the surgery may also improve the prognosis. Radiotherapy is not efficient because the tumor is not differentiated.
A good washing of the peritoneal cavity is useful, but the lesions frequently come back. The surgery must remove the appendix, and sometimes even a the right part of the colon, the uterus and the ovaries.
Is Pseudomyxoma Peritonei an inherited disorder?
There are no genetic factors known to be associated with pseudomyxoma peritonei (PMP), and to our knowledge, no familial cases of PMP have been reported. Therefore, family members of people with PMP are not thought to be at an increased risk for developing the condition.