Pseudomyxoma peritonei

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Pseudomyxoma peritonei (PMP) is a rare medical condition characterized by the accumulation of mucinous tumor cells within the peritoneal cavity. This condition is often referred to as "jelly belly" due to the gelatinous nature of the mucinous material.

Etiology

Pseudomyxoma peritonei typically originates from a mucinous tumor of the appendix, although it can also arise from other gastrointestinal or ovarian sources. The primary tumor releases mucin-producing cells into the peritoneal cavity, where they proliferate and produce large amounts of mucin.

Pathophysiology

The mucinous material accumulates within the peritoneal cavity, leading to increased abdominal girth and discomfort. The mucin can cause adhesions and obstruction of the bowel, leading to significant morbidity. The condition progresses slowly but can be life-threatening if not managed appropriately.

Clinical Presentation

Patients with pseudomyxoma peritonei often present with:

• Abdominal distension
• Abdominal pain
• Changes in bowel habits
• Weight loss
• Nausea and vomiting

Diagnosis

The diagnosis of pseudomyxoma peritonei is typically made through a combination of imaging studies and histopathological examination. CT scans and MRI are commonly used to visualize the extent of mucinous deposits. A definitive diagnosis is made through biopsy and examination of the mucinous material.

Treatment

The primary treatment for pseudomyxoma peritonei is cytoreductive surgery combined with hyperthermic intraperitoneal chemotherapy (HIPEC). This approach aims to remove as much of the tumor burden as possible and deliver high concentrations of chemotherapy directly to the peritoneal cavity to eradicate residual disease.

Prognosis

The prognosis for patients with pseudomyxoma peritonei varies depending on the extent of disease and the success of surgical intervention. Early diagnosis and aggressive treatment are associated with improved outcomes.

See Also

• Peritoneal carcinomatosis
• Appendiceal cancer
• Mucinous adenocarcinoma
• Hyperthermic intraperitoneal chemotherapy