Background:

Pseudomyxoma peritonei is characterized by disseminated intraperitoneal mucinous tumors and mucinous ascites in the abdomen; with incidence of 1/1,000,000, it predominates in females; 30-50% of patients have progressive abdominal ascites, accompanied by abdominal pain, weight loss and dyspnea; in 90% of patients, the primary lesion is a mucinous tumor of appendix. Computed tomography and histopathology are the most reliable diagnostic modalities. Current treatment is debulking surgery combined with hyperthermic intraperitoneal chemotherapy, followed by intravenous chemotherapy.

Clinical case:

A 31 years old male with previous appendectomy eight years ago, with increasing of abdominal girth of two months, 8 kg weight loss and progressive dyspnea. On examination: globular abdomen by ascites, decreased peristalsis and no evidence of acute abdomen; he subjected to laparotomy with removal of six liters of ascitic fluid and debulking surgery accompanied by hyperthermic chemotherapy. 27 months after diagnosis the patient is rated in 1 of functional ECOG scale.

Conclusions:

Pseudomyxoma peritonei is a rare disease, its treatment includes debulking surgery combined with intraperitoneal hyperthermic chemotherapy.