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Pseudomyxoma peritonei: a review of 62 cases
S van Ruth1, Y I Z Acherman, M J van de Vijver
1Department of Surgical Oncology, The Netherlands Cancer Institute/Antoni van Leeuwenhoek Hospital, Amsterdam, The Netherlands. s.v.ruth@nki.nl
Aim:
Pseudomyxoma peritonei (PMP) is a rare disease characterized by the abundance of mucus in the abdomen without extra-peritoneal growth.
Methods:
Our patients with PMP have been treated with cytoreduction and hyperthermic intraperitoneal chemotherapy since 1996. The clinical and histopathological features of PMP and the relation of these features with disease-free interval and survival were assessed.
Results:
Sixty-two patients with PMP (24 M/38 F) were studied. Adenomatous mucosal changes were present in 31 patients. In females, the ovaries were normal in 5 patients and pseudomyxoma ovarii was present in 20 patients. Patients with minimal atypia and with 1% focal proliferation or less (n=38) had a better survival (p=0.0008) than those with more focal proliferation (n=14).
Conclusion:
In most patients with PMP the appendix is affected; in females the ovaries are usually also involved. Focal proliferation appears to be a prognostic factor.
Insights
Pseudomyxoma peritonei (PMP) is a rare abdominal mucus disease. Focal proliferation in PMP patients indicates better survival rates, suggesting it as a key prognostic factor.
Area of Science:
- Oncology
- Gastroenterology
- Surgical Pathology
Background:
- Pseudomyxoma peritonei (PMP) is a rare clinical condition characterized by abundant mucus accumulation within the peritoneal cavity.
- PMP typically originates from appendiceal mucinous neoplasms, with potential ovarian involvement in females.
Purpose of the Study:
- To evaluate clinical and histopathological features of PMP.
- To assess the relationship between these features and disease-free interval and survival.
- To identify prognostic factors in PMP patients.
Main Methods:
- Retrospective analysis of 62 patients with PMP treated with cytoreduction and hyperthermic intraperitoneal chemotherapy since 1996.
- Clinical and histopathological data were collected and analyzed.
- Statistical methods were used to correlate features with disease outcomes.
Main Results:
- Appendiceal involvement was common in PMP patients.
- Ovarian involvement (pseudomyxoma ovarii) was observed in 20 out of 25 female patients.
- Patients with minimal atypia and less than 1% focal proliferation (n=38) demonstrated significantly better survival compared to those with higher focal proliferation (n=14) (p=0.0008).
Conclusions:
- The appendix is frequently affected in PMP, and the ovaries are commonly involved in females.
- The degree of focal proliferation is a significant prognostic factor for survival in PMP.
- Cytoreduction and hyperthermic intraperitoneal chemotherapy are established treatment modalities for PMP.