Pseudomyxoma peritonei (PMP) is very rare. Approximately two people in 1 million will develop PMP, which grows out of the cells of the appendix as a benign tumour at first, but spreads as a low grade malignancy as it progresses. The cancer produces a jelly-like liquid called mucin, which eventually breaks out from the appendix and spreads into the peritoneal cavity. The peritoneum lines the inside of the abdomen, covering and protecting organs such as the ovaries, uterus, and bowels. The tumour affects men and women equally, but in women, it is usually associated with ovarian cancer which could be a metastasis of the primary appendiceal tumour.
Symptoms of PMP can include hernia (a bulge on the abdominal wall), loss of appetite, unexplained weight gain, pelvic pain, and/or changes in bowel habits. All of these symptoms will depend on where the tumour is growing and what organs it is affecting.
PMP is diagnosed through a battery of tests to eliminate the possibility of other more common diseases. These tests can include:
- CT scan.
- Pelvic or abdominal ultrasound.
- Drainage of fluid from the abdomen.
Treatment for this tumour usually consists of two types of surgery: Cytoreductive surgery with hyperthermic intraperitoneal chemotherapy (HIPEC) and debulking surgery.
HIPEC involves a solution containing chemotherapy that is heated to a temperature greater than body temperature. Before HIPEC is administered, the surgeon removes all visible tumours. This is known as cytoreductive surgery. Following cytoreductive surgery, the surgeon will administer HIPEC treatment directly into the abdominal cavity in the operative setting.
Debulking surgery is performed when other treatments are insufficient, and involves the removal of the affected peritoneum, part of the bowels and, in women, the uterus and ovaries.