[The clinical and pathological characteristics and prognosis of pseudomyxoma peritonei].
OBJECTIVE
To investigate the clinical and pathological characteristics, treatment measures and prognosis of pseudomyxoma peritonei (PMP).


METHODS
The clinical records and follow-up data of the patients with PMP were retrospectively analyzed. Survival analysis (Kaplan-Meier method and Cox regression) was used to investigate the prognosis.


RESULTS
Thirty-three patients (twenty-two women, eleven men) have a median interval between onset and definite diagnosis of 12 months with an average age of 50. The main clinical presentation includes bloating, abdominal mass and abdominal distention. A delay in diagnosis is common and many patients are labeled as other diseases (84.8%). Positive results were often found in the assistant examinations such as erythrocyte sedimentation rate, gastrointestinal tumor markers (carcinoembryonic antigen, carcinomatous antigen 19-9 and so on), imaging (B ultrasound and computer tomography) and abdominal paracentesis which was usually unsuccessful. The most frequently practiced approach is debulking surgery with the main assistant means of chemotherapy which was lack of the uniform rationale. Benign type of pathological samples accounts for 66.7% of all, while mid-type 21.2% and malignant 12.1%. The general median survival time is 70 months with a follow-up rate of 79%. The pathological type and chemotherapy are main factors which influence the cumulative survival rate (P < 0.05). Up to the end of 2004, sixteen patients died of various complications, multiple organ failure and severe infection, while ten patients survival and seven patients lost.


CONCLUSIONS
PMP is a rare condition of borderline malignancy with its unique clinical and pathological characteristics. Gastrointestinal tumor markers, imaging and abdominal paracentesis are very important to the diagnosis. The existing treatment measures need to be improved further. The pathological type and chemotherapy are main factors which influence the prognosis.
Song
Zhi-qiang
ZQ
Department of Gastroenterology, Peking Union Medical College Hospital, Peking Union Medical College, Chinese Academy of Medical Sciences, Beijing 100730, China. szqinpumch@yahoo.com.cn
Wang
Wen-ze
WZ
Lu
Xing-hua
XH
Ke
Mei-yun
MY
Sun
Xiao-hong
XH
Cui
Quan-cai
QC
D000293:Adolescent; D000328:Adult; D000368:Aged; D005260:Female; D005500:Follow-Up Studies; D006801:Humans; D008297:Male; D008875:Middle Aged; D010534:Peritoneal Neoplasms / Q000175:diagnosis* / Q000473:pathology* / Q000628:therapy; D011379:Prognosis; D011553:Pseudomyxoma Peritonei / Q000175:diagnosis* / Q000473:pathology* / Q000628:therapy; D012189:Retrospective Studies; D016019:Survival Analysis