Retrospective study of different methods for managing Egyptian patients with pseudomyxoma peritonei: feasibility and overall outcome

Introduction
Pseudomyxoma peritonei (PMP) is a rare disease with poor outcome when not treated properly and is characterized by mucinous ascites and peritoneal implants. Treatment for PMP is variable, both because of the rarity of the disease and because of its often slow-growing nature. There is no consensus regarding the proper management of aggressive cases. Our study was designed to analyze the symptoms and signs of PMP, as well as the diagnostic tools, and evaluate the effect of treatment and factors influencing postoperative recurrence of and survival from PMP in Egyptian patients.
Patients and Methods
We reviewed consecutive cases of PMP that presented at the surgical department of the Main Alexandria University Hospital from January 1990 to December 2012.
Results
This study included 62 patients with PMP: 43 were women (69%) and 19 were men (31%). Their mean age at the time of diagnosis was 47.3 ± 11.6 years (median 49, range 29-67). The predominance of women was statistically significant ( = 0.08). A total of 69 surgical procedures had been performed in 46 cases, including 46 primary operations, 10 secondary operations, one tertiary cytoreduction and peritonectomy, and 12 debulking procedures for recurrence.
Conclusion
Surgical debulking is the standard treatment for PMP in primary and recurrent tumors. Intraperitoneal chemotherapy either intraoperative or postoperative is accompanied by better disease-free survival and overall survival. Referring of patients to specialized centers that treat these patients on a regular basis is essential to prevent high morbidity and mortality. Recurrence is common and requires reoperation with or without adjuvant chemotherapy.