PSEUDOMYXOMA PERITONEI Dr.Salahaldeen Abdulnabi Gastroenterology & Hepatology Center

Definition A rare condition in which diffuse collection of gelatinous fluid are associated with mucinous implants on peritoneal surfaces and omentum Involve 2 in laprotomies. 75% are women at 45-75y of age.

Pathogenesis Recent immunocytologic & molecular studies suggest that the appendix is the site of origin for the overwhelming majority of cases, and to a lesser extent the ovary. Ps.Per. caused by neoplastic mucus-secreting cells within the peritoneum,these cells may be difficult to classify as malignant because they may be spares,widely scattered,and have a low-grade cytologic appearance. Lymphatic, hematogenous & extraperitoeal spread is rare.

Signs & Symptoms Presenting symptoms, painless abdominal distention, pain, or a mass. Mucin may accumulate intraperitoneally many years after resection of an ovarian mass. Primary Ps. Per. usually does not cause abdominal organ dysfunction, however, uretral obstruction, bowel obstruction, venous return obstruction, malnutrition & death.

Diagnostics Abdominal taping Dry. CT abdomen is the preffered imaging. At surgery a variable volume of mucinous ascites is found together with tumor deposits involving the right hemidiaphragm,right retro- hepatic space, left paracolic gutter, ligament of Treitz, & ovareis in women. peritoneal surface of the bowel are usually free of tumor.

Management Thourogh surgical debulking is the mainstay of treatment. all gross disease & the omentum should be removed. If not done previouslly appendectomy is routinely performed. Hysterectomy with bilateral salpingo- oophorectomy is performed in women.

Management The correct treatment for mucinous cyst- adenocarcinoma of the appendix is right hemicolectomy with aggressive debulking in the presence of Ps.Per. Intraperitoneal hyperthermic chemotherapy is advocated as a standared adjuvent to radical cytoreductive surgery. Its degree of malignant potential is variable; about % of patients live 5y.

Management Is a long, tedious procedure with operative time of min reported, with morbidity of 38% & mortality of 6% are high. 5y survival of between 53-78% after this Tr. Ps.Per. Is a disease that progress slowly and in which recurrences may take years to develop or become symptomatic. The Mayo clinic series,76% of patients develop recurrence within the abdomen.

Management Recurrences are usually treated by additional surgery. It is important to note that surgery for recurrent disease is usually difficult and is associated with an increased incidence of unintentional enterotomies, anastomatic leak and fistulas.

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