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Pseudomyxoma peritonei
1Department of General Surgery, St James's University Hospital, Leeds, UK.
Background:
Pseudomyxoma peritonei is an unusual condition in which gelatinous fluid collections are associated with mucinous implants on the peritoneal surfaces and omentum. The pathological origin and ideal treatment of the condition are subjects of debate.
Methods:
An unrestricted Medline search over 1986-1997 was performed for pseudomyxoma peritonei.
Results And Conclusions:
There is increasing evidence that pseudomyxoma peritonei is a neoplastic condition which usually arises from a primary adenoma or adenocarcinoma of the appendix. Reported series include a spectrum of pathological lesions, from entirely benign ruptured mucocele to advanced carcinoma. This, and the rarity of the condition, limit the conclusions that can be drawn regarding its treatment and prognosis. Most authorities agree that a thorough surgical debulking should be made. In most cases this will be a difficult and time-consuming undertaking, possibly requiring cooperation between two or more specialists and consideration of delivering intraperitoneal adjuvant therapy during or immediately after surgery. Treatment therefore requires a planned approach with accurate preoperative assessment of the diagnosis and the extent of the condition. There is some largely anecdotal evidence in favour of intraperitoneal chemotherapy and radioisotope treatment. Ultraradical surgery, with heated intraoperative and further postoperative chemotherapy, is strongly advocated by one group but remains contentious. The majority of patients will eventually suffer recurrence. The 5-year survival rate ranges from 53 to 75 per cent, but outcomes vary widely between relatively benign and malignant subgroups.
Insights
Pseudomyxoma peritonei is a neoplastic condition, often originating from the appendix. Treatment involves extensive surgery and potentially adjuvant therapies, though recurrence is common.
Area of Science:
- Oncology
- Gastroenterology
- Pathology
Background:
- Pseudomyxoma peritonei involves gelatinous fluid and mucinous implants on peritoneal surfaces.
- Its pathological origin and optimal treatment remain subjects of debate.
Purpose of the Study:
- To review the literature on pseudomyxoma peritonei.
- To discuss the neoplastic origin, treatment strategies, and prognosis of this rare condition.
Main Methods:
- An unrestricted Medline search was conducted for pseudomyxoma peritonei literature from 1986 to 1997.
Main Results:
- Evidence suggests pseudomyxoma peritonei is neoplastic, often arising from appendiceal adenoma or adenocarcinoma.
- Pathological findings range from benign mucocele to advanced carcinoma.
- Surgical debulking is generally recommended, often requiring multidisciplinary teams and adjuvant therapies.
Conclusions:
- Pseudomyxoma peritonei is a spectrum of neoplastic disease, typically appendiceal in origin.
- Treatment requires planned surgical debulking, possibly with intraperitoneal chemotherapy or radioisotope therapy.
- Recurrence is frequent, with 5-year survival rates varying from 53-75% based on disease subgroup.