Listed among other “orphan” diseases in the National Association of Rare Disorders database, DPAM affects fewer than 200,000 Americans.
What is DPAM?
DPAM is a type of pseudomyxoma peritonei (PMP), a rare disease characterized by mucus-secreting tumors in the abdomen. It develops after a small growth (polyp) in the appendix bursts through the appendix wall, spreading tumor cells.
Who develops DPAM?
The underlying cause of DPAM is unclear, but appendix cancer is the most common source of the tumors that cause PMP. There are no known genetic, familial or environmental risk factors associated with the disease.
What are the symptoms of DPAM?
Symptoms generally progress slowly and may not be recognized initially. As tumor cells accumulate, the abdominal area becomes swollen and digestive function becomes impaired. Other symptoms may include:
increased abdominal size (so-called “jelly belly”)
hernia symptoms in men
ovarian cyst or tumor symptoms in women
How is DPAM diagnosed?
For many patients, DPAM is found during an unrelated surgical procedure. Tests to confirm the diagnosis include computed tomography (CT) scans, exploratory laparoscopy and blood tests.
How is DPAM treated?
Treatment for DPAM typically includes surgery and heated intraperitoneal chemotherapy.