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.