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Therapy of pseudomyxoma peritonei of appendiceal origin--surgical resection and intraperitoneal chemotherapy
W P Hosch1, J Rudi, W Stremmel
1Department of Gastroenterology, University of Heidelberg.
Abstract:
Pseudomyxoma peritonei (PMP) is rare clinical entity presenting with mucus-producing lesions on the peritoneal surface. PMP is found in middle-aged or older patients in approximately two out of 10,000 laparotomies. Most of the reported cases are due to a primary process located in either appendix or the ovaries. PMP presents with an insidious onset of symptoms, and is characterized by long-term survival with good general health and absence of visceral invasion or distant metastasis. The treatment of choice for PMP is surgical resection and the removal of free mucus. Despite a high rate of disease recurrence, adjuvant or palliative chemotherapy has not yet been clearly established. In our opinion, intraperitoneal application may be more effective than a systemic regimen because only local treatment can provide sufficient concentrations of chemotherapeutic agents. In view of the lack of standardized treatment regimens and based on our experience, we recommend intraperitoneal chemotherapy with 5-FU and mitomycin C or cisplatin. We report ten cases of PMP of appendiceal origin and present a review of the literature on this disease and its treatment.
Insights
Pseudomyxoma peritonei (PMP) is a rare condition causing mucus on the peritoneum, often from the appendix. Intraperitoneal chemotherapy with 5-FU, mitomycin C, or cisplatin is recommended for appendiceal PMP due to high recurrence rates.
Area of Science:
- Oncology
- Gastroenterology
- Surgical Pathology
Background:
- Pseudomyxoma peritonei (PMP) is a rare condition characterized by mucinous lesions on the peritoneal surface.
- Typically affects middle-aged or older adults, with an incidence of approximately 2 in 10,000 laparotomies.
- Most cases originate from the appendix or ovaries, presenting insidiously with long-term survival and minimal distant spread.
Purpose of the Study:
- To review the literature on Pseudomyxoma peritonei (PMP) and its treatment.
- To present ten cases of PMP of appendiceal origin.
- To recommend effective treatment strategies for PMP, particularly intraperitoneal chemotherapy.
Main Methods:
- Literature review on Pseudomyxoma peritonei (PMP) and its management.
- Analysis of ten cases of PMP with appendiceal origin.
- Evaluation of treatment efficacy, focusing on surgical resection and chemotherapy.
Main Results:
- Surgical resection and removal of mucinous material are the primary treatments for PMP.
- Disease recurrence is common, and adjuvant/palliative chemotherapy regimens are not well-established.
- Intraperitoneal chemotherapy may offer superior local drug concentrations compared to systemic administration.
Conclusions:
- Intraperitoneal chemotherapy with 5-FU, mitomycin C, or cisplatin is recommended for appendiceal PMP.
- This approach is based on the potential for higher local drug concentrations and the experience from ten reported cases.
- Further research into standardized chemotherapy regimens for PMP is warranted.
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