PSEUDOMYXOMA PERITONEI - A REVIEW OF 23 CASES

Objective: To review the experience at the Massachusetts General and B righam and Women's Hospitals with 23 women treated for pseudomyxoma pe ritonei between 1961 and 1991. Methods: Patients were identified retro spectively from the tumor registry at the Massachusetts General and Br igham and Women's Hospitals, and all charts were reviewed. The median follow-up interval was 2.5 years (range 3 months to 31 years). Results : The mean age at diagnosis was 58 years (range 26-76). Pseudomyxoma p eritonei was found in association with ten (44%) ovarian tumors of bor derline malignancy, nine (39%) ovarian cystadenocarcinomas, and four ( 17%) appendiceal cystadenocarcinomas. Three patients had synchronous t umors in the ovary and appendix. All patients underwent surgical stagi ng and cytoreduction. Eleven patients received postoperative therapy a nd, of these, nine developed a recurrence; 12 patients received no fur ther therapy and, of these, three developed a recurrence. However, the se groups were not pathologically comparable. With respect to survival , of the ten patients with borderline malignancies, seven had no evide nce of disease, one was alive with disease, and two died of disease. F or the nine patients with ovarian cystadenocarcinomas, three had no ev idence of disease, one was alive with disease, and five died of diseas e (median time to death 18 months). For the four patients with appendi ceal carcinomas, two had no disease, one was alive with disease, and o ne died with disease. Among all 23 patients, 12 (52%) developed a recu rrence, with a range of time to first recurrence of 3 months to 19 yea rs. Eight women required at least one additional laparotomy because of accumulation of gelatinous material. Conclusions: Although pseudomyxo ma peritonei is associated with borderline and well-differentiated tum ors, recurrence is common and the prognosis after recurrence is guarde d. Involvement of the appendix is common; therefore, appendectomy is i ndicated when pseudomyxoma is encountered. To date, surgery has been t he only effective therapy for this disease, and adjuvant therapy has n ot been shown conclusively to be of benefit.