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Published on: December 9, 2022
Pseudomyxoma peritonei: a clinicopathologic analysis and follow-up of 21 patients
J F Varona1, J M Guerra, J Salamanca
1Department of Internal Medicine, 12 de Octubre University Hospital of Madrid, Spain. jfva_varona@yahoo.com
Background/Aims:
Pseudomyxoma peritonei is an uncommon disease characterized by the presence of mucinous peritoneal implants associated with an abdominal neoplasm. Our objective is to consider the characteristics of this entity in our western Mediterranean urban population.
Methodology:
All cases diagnosed with pseudomyxoma peritonei by our hospital during a period of 16 years were reviewed. Data from their clinical records and the biopsy samples were analyzed.
Results:
We found 21 cases of pseudomyxoma peritonei with a male/female ratio of 10/11 and a mean age of 59 years. The predominant presentation symptom was abdominal pain (17 cases, 6 of them with acute abdomen). The most frequent primary site of origin of the pseudomyxoma was the appendix (10 cases). The histologic diagnosis was malignant (associated with carcinoma) in 17 cases and indeterminate behavior in 4. The follow-up was available for 15 patients (mean follow-up of 41 months), while six patients have been lost. Nine patients have died during the follow-up and the other 6 patients are still alive after follow-up.
Conclusions:
Laparotomy is the main tool for diagnosing pseudomyxoma peritonei. The appendix is the most frequent primary site of origin of pseudomyxoma peritonei, followed by bowel; the latter being more important than previously described. In most cases the histology is malignant. The prognosis is bad with a mortality greater than 60% at 5 years.
Insights
Pseudomyxoma peritonei, a rare condition of mucinous peritoneal implants, most often originates in the appendix. This study highlights its characteristics in a Mediterranean population, revealing a poor prognosis with over 60% mortality within 5 years.
Area of Science:
- Gastroenterology
- Oncology
- Pathology
Background:
- Pseudomyxoma peritonei is a rare condition involving mucinous peritoneal implants linked to abdominal neoplasms.
- Understanding its characteristics in specific populations is crucial for diagnosis and management.
Purpose of the Study:
- To characterize pseudomyxoma peritonei within a western Mediterranean urban population.
- To analyze clinical presentation, primary sites, histology, and prognosis.
Main Methods:
- Retrospective review of 21 cases diagnosed over 16 years.
- Analysis of clinical records and biopsy samples.
Main Results:
- The study identified 21 cases with a near equal male/female ratio and a mean age of 59.
- Abdominal pain was the most common symptom, and the appendix was the most frequent primary site (10 cases).
- Histology was malignant in 17 cases, with a 5-year mortality rate exceeding 60%.
Conclusions:
- Laparotomy is key for diagnosing pseudomyxoma peritonei.
- The appendix is the most common primary site, with bowel involvement being more significant than previously reported.
- Pseudomyxoma peritonei generally presents as malignant with a poor prognosis.
