Pseudomyxoma peritonei of 92 Chinese patients: clinical characteristics, pathological classification and prognostic factors.
OBJECTIVE
To assess the clinicopathologic features and its relationship with prognosis of pseudomyxoma peritonei (PMP) in Chinese patients.


METHODS
The clinicopathologic features and follow-up data of 92 patients with PMP were reviewed and retrospectively analyzed. The cases were categorized into three groups: disseminated peritoneal adenomucinosis (DPAM), peritoneal mucinous carcinomatosis (PMCA), and peritoneal mucinous carcinomatosis with intermediate or discordant features (PMCA-I/D). The log-rank test was used to analyze survival for each group and various clinicopathological parameters. Multivariate Cox proportional-hazard models were constructed to determine the important factors associated with survival.


RESULTS
The median age at diagnosis was 51.9 years (range: 22-76 years). The median follow up was 124 mo. The 3-, 5- and 10-year survival rates were 74.0%, 67.4% and 49.1%, respectively. There were 49 (53.2%) patients with DPAM, 26 (28.3%) with PMCA-I and 17 (18.5%) with PMCA. Patients with DPAM, PMCA-I/D and PMCA exhibited statistically significant difference in survival (P = 0.001). The 3 year survival for DPAM, PMCAI/D and PMCA was 97.0%, 80.0% and 67.0%, respectively; the 5 year survival was 80.0%, 67.0% and 50.0%, respectively; and the 10 year survival was 65.0%, 28.0% and 14.0%, respectively. Survival rate was significantly lowest in patients < 40 age years of age (P = 0.011). Appendiceal tumor and extra-ovarian parenchymal organ involvement were significantly related to overall survival. Patients with appendiceal mucinous adenocarcinoma (MACA) showed the significantly poorer prognosis (P = 0.011). Multivariate analysis showed that pathological classification, age, appendiceal tumor were significant related to overall survival.


CONCLUSIONS
The clinical process "PMP" should be pathologically classified into DPAM, PMCA and PMCA-I/D. Pathological classification, age, appendiceal MACA are survival independent predictors in Chinese patients with PMP.
Department of Pathology, the General Hospital of the People's Liberation Army, Beijing 100853, China.
Ai-Tao
AT
Guo
Yan-Mi
YM
Li
Li-Xin
LX
Wei
D000328:Adult; D000367:Age Factors; D000368:Aged; D001063:Appendiceal Neoplasms / Q000145:classification / Q000175:diagnosis* / Q000401:mortality / Q000473:pathology; D044466:Asian People; D002681:China / Q000453:epidemiology; D005260:Female; D006801:Humans; D053208:Kaplan-Meier Estimate; D008297:Male; D008875:Middle Aged; D015999:Multivariate Analysis; D010534:Peritoneal Neoplasms / Q000145:classification / Q000175:diagnosis* / Q000401:mortality / Q000473:pathology; D011379:Prognosis; D016016:Proportional Hazards Models; D011553:Pseudomyxoma Peritonei / Q000145:classification / Q000175:diagnosis* / Q000401:mortality / Q000473:pathology; D012189:Retrospective Studies; D018570:Risk Assessment; D012307:Risk Factors; D009626:Terminology as Topic; D013997:Time Factors; D055815:Young Adult