A Clinical Review of Pseudomyxoma Peritonei

Jung, Jun Keun; Chon, Chae Yoon; Cho, Hyeon Geun; Kim, Kyung Chul; Park, Hyo Jin; Chung, Jun Pyo; Lee, Kwan Sik; Lee, Sang In; Kang, Jin Kyung; Park, In Suh

Abstract

BACKGROUND AND AIMS: Pseudomyxoma peritonei is a rare malignancy in which the peritoneal surfaces and omentum are involved with diffuse gelatinous mucinous implants. It arises from mucinous neoplasma of the appendix and ovary, and from mucin-producing cells disseminted within the peritoneal cavity. A large scale collective review of pseudomyxnma peritonei has not been reported at present in Korea, The aims of this study were to analyze the natural history of the disease, to examine the clinical and pathologic features that might act as prognostic indicators, and to review our experience with available therapeutic modalities.
METHODS
Thirty-six patients (10 men, 26 women) were included. The clinical data were collected retrospectively. Information on both survival and tumor recurrence was described using the Kaplan-Meier method. Univariate assessments of potential risk factors with respect to both recurrence and survival were performed for categoric variables.
RESULTS
There was a preponderance of women (M:F=l:2.6), the mean patient age at presentation was 54.9 years, and the median duration of symptoms was 2 months. The most common presenting symptoms were abdominal pain (50%) and a4dominal fullness (47%). The site of tumor origin was most commonly the ovary (42%). The pathologic diagnosis was adenocarcino-mas (50%), and cystadenocarcinomas (47%). Surgery was performed in 26 patients (72%); 50% of these patients also received postoperative adjuvant chemotherapy. The median survival time was were 25.3 months for the entire group; 29.5 months for surgery 1 (I,3 months for chernotherapy, and 7.5 months for na treatent group respectively. Postoperative adjuvant chernotherapy did not improve the surate. The recurrence rate of disease after curative resection was 56%. Weight loss (p=0.013) and residual tumor after surgery (p=0.026) were an adversely affecting factors on survival,
CONCLUSIONS
Pseudomyxoma peritonei is a progressive and frequently relapsed malignancy, but aggressive and repeated debulking surgery is recommeded.