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Pseudomyxoma peritonei--a revisit: report of 2 cases and literature review
Chunyanca Li1, Rani Kanthan, S C Kanthan
1Department of Pathology, Royal University Hospital, 103, Hospital Drive, Saskatoon, Saskatchewan, SK S7N OW8, Canada. chunyanca@yahoo.ca
Background:
Pseudomyxoma peritonei (PMP) is a rare, chronic, relapsing, diagnostically challenging and poorly understood disease characterized by disseminated mucinous ascites and peritoneal implants.
Case Presentation:
We report two cases of PMP that represent the two biological variants of disseminated peritoneal adenomucinosis (DPAM)--the benign variant and the peritoneal mucinous carcinomatosis (PMCA)--the malignant variant, both of which were characterized by multiple relapses and progression of the disease despite aggressive management.
Conclusion:
Even with a better understanding and recent advances in the management of these cases, PMP remains an enigmatic disease with a protracted clinical course characterized by multiple recurrences despite surgery and/or chemotherapy. Recognition of PMP as a delayed consequence years later should alert all surgeons to be extremely vigilant when treating mucinous neoplasms of the appendix, with special care being directed towards adequate excision and thorough debridement at the initial diagnosis.
Insights
Pseudomyxoma peritonei (PMP) is a rare, challenging disease. This report details two PMP cases, highlighting its relapsing nature and the need for surgical vigilance with mucinous appendiceal neoplasms.
Area of Science:
- Oncology
- Gastroenterology
- Surgical Pathology
Background:
- Pseudomyxoma peritonei (PMP) is a rare, chronic, and poorly understood condition.
- Characterized by disseminated mucinous ascites and peritoneal implants, PMP presents diagnostic challenges.
- It follows a relapsing course, often with delayed diagnosis.
Purpose of the Study:
- To present two cases of Pseudomyxoma peritonei (PMP).
- To illustrate the two biological variants of PMP: disseminated peritoneal adenomucinosis (DPAM) and peritoneal mucinous carcinomatosis (PMCA).
- To emphasize the challenges in managing recurrent PMP.
Main Methods:
- Case report of two patients with Pseudomyxoma peritonei.
- Detailed clinical course including diagnosis and management strategies.
- Pathological review of both benign (DPAM) and malignant (PMCA) variants.
Main Results:
- Both reported PMP cases exhibited multiple relapses and disease progression.
- Despite aggressive management including surgery and chemotherapy, recurrence was observed.
- The cases highlight the distinct biological behaviors of DPAM and PMCA variants.
Conclusions:
- Pseudomyxoma peritonei (PMP) remains an enigmatic disease with a protracted clinical course.
- Multiple recurrences are common despite optimal management.
- Surgeons must be vigilant with mucinous neoplasms of the appendix, ensuring adequate excision and debridement due to PMP's potential as a delayed consequence.