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Pseudomyxoma peritonei of appendiceal origin. The Memorial Sloan-Kettering Cancer Center experience
J W Smith1, N Kemeny, C Caldwell
1Department of Surgery, Memorial Sloan-Kettering Cancer Center, New York, NY 10021.
Background:
Pseudomyxoma peritonei is a rare clinical entity in which the peritoneal surfaces and omentum are involved with diffuse gelatinous mucinous implants. It originates from ruptured mucinous tumors of the appendix or ovary.
Methods:
The authors examined the experience with 34 patients with pseudomyxoma peritonei seen at Memorial Sloan-Kettering Cancer Center from 1952-1989. Of these, 17 cases were identified to be of appendiceal origin.
Results:
All patients underwent celiotomy and cytoreduction. The median survival time from diagnosis was 75 months. It was found that long-term survival can be achieved by operation alone. When conditions do recur, chemotherapy may be valuable.
Conclusions:
Pseudomyxoma peritonei of appendiceal origin is a rare low-grade malignancy. Initial treatment consists of cytoreduction in an attempt to render the patient locally disease-free. Long-term survival can be obtained by operation alone, even if gross disease is present at the end of the procedure. Systemic chemotherapy should be reserved for patients with proven recurrence.
Insights
Pseudomyxoma peritonei (PMP) is a rare malignancy originating from appendiceal or ovarian tumors. Surgical cytoreduction alone can achieve long-term survival for PMP patients, with chemotherapy reserved for recurrent disease.
Area of Science:
- Oncology
- Gastroenterology
- Surgical Oncology
Background:
- Pseudomyxoma peritonei (PMP) is a rare condition characterized by gelatinous mucinous implants on peritoneal surfaces and omentum.
- PMP typically originates from ruptured mucinous tumors of the appendix or ovary.
Purpose of the Study:
- To review the experience with pseudomyxoma peritonei (PMP) at Memorial Sloan-Kettering Cancer Center.
- To evaluate treatment outcomes and survival for PMP patients, particularly those of appendiceal origin.
Main Methods:
- Retrospective review of 34 patients with PMP treated between 1952 and 1989.
- Analysis of 17 cases specifically identified as having appendiceal origin.
- All patients underwent celiotomy and cytoreductive surgery.
Main Results:
- The median survival time from diagnosis was 75 months.
- Long-term survival was achievable with surgical operation alone.
- Chemotherapy demonstrated value in managing recurrent disease.
Conclusions:
- Pseudomyxoma peritonei of appendiceal origin is a rare, low-grade malignancy.
- Initial treatment focuses on cytoreduction to achieve a locally disease-free state.
- Surgical intervention alone can lead to long-term survival, even with residual gross disease; systemic chemotherapy is indicated for recurrence.