Long-term survival following treatment of pseudomyxoma peritonei: an analysis of surgical therapy.
BACKGROUND
Pseudomyxoma peritonei (PMP) is a clinical syndrome with a poorly defined natural history. Relative contributions of tumor biology, patient selection, and the extent of treatment on ultimate outcome are not well characterized.


METHODS
Patients treated at the Memorial Sloan-Kettering Cancer Center between 1980 and 2002 with a diagnosis of PMP were identified. Patient characteristics, pathologic features, and details of treatment were analyzed retrospectively.


RESULTS
The 97 patients included in this study underwent a mean 2.2 +/- 0.1 operations (range, 1-6). Although complete cytoreduction was achieved in 55% (53/97), disease recurred in 91% (48/53) of patients. The median disease-free interval after complete cytoreduction was 24 months. The median overall survival was 9.8 years and was independently associated with low-grade pathologic subtype (P < 0.001) and the ability to achieve complete cytoreduction (P < 0.001). Ten-year survival was attained in 21% (20/97) of the patients, of which 90% (18/20) had low-grade pathologic features. At the time of death or completion of follow-up, only 12% (12/97) of the patients were disease free.


CONCLUSIONS
Outcome in patients with PMP is strongly associated with tumor biology. Although improved survival is associated with low-grade pathology and tumors amenable to complete cytoreduction, recurrence of PMP is common. Treatment may be beneficial, particularly in controlling symptoms, but absolute cure, defined as a prolonged disease-free state, is uncommon.
Miner
Thomas J
TJ
Memorial Sloan-Kettering Cancer Center, New York, New York 10021 USA.
Shia
Jinru
J
Jaques
David P
DP
Klimstra
David S
DS
Brennan
Murray F
MF
Coit
Daniel G
DG
D002288:Adenocarcinoma, Mucinous / Q000401:mortality / Q000473:pathology / Q000601:surgery; D000328:Adult; D000368:Aged; D000369:Aged, 80 and over; D018572:Disease-Free Survival; D005260:Female; D006801:Humans; D008297:Male; D008875:Middle Aged; D018579:Patient Selection; D010534:Peritoneal Neoplasms / Q000401:mortality / Q000473:pathology / Q000601:surgery*; D011553:Pseudomyxoma Peritonei / Q000401:mortality / Q000473:pathology / Q000601:surgery*