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Published on: December 9, 2022
Pseudomyxoma peritonei secondary to epithelial appendicular neoplasms. Experience in a non-specialised centre
J Ruiz-Tovar1, V Morales Castiñeiras, D García Teruel
1Department of Surgery, University Hospital Ramón y Cajal, Madrid, Spain. jruiztovar@gmail.com
Introduction:
Pseudomyxoma peritonei is an infrequent entity, defined by collections of gelatinous material in the abdomen and pelvis and mucinous implants on peritoneum, secondary to the rupture of a mucinous lesion, usually of ovarian or appendiceal origin.
Materials And Methods:
We present our experience of 11 cases (6 males and 5 females) diagnosed with pseudomyxoma peritonei secondary to epithelial appendicular neoplasms over 27 years. The mean age of the patients was 68 years. Clinical manifestations were abdominal distension (55%), right lower quadrant pain (45%) suggesting acute appendicitis and constitutional syndrome (36%). An abdominal mass was detected at physical examination in 4 patients. CT scan revealed a tumour in right iliac fossa in 4 patients, peritoneal enlargement in 1 and a liquid collection in 1. Preoperative diagnosis was acute abdomen in 5 patients, peritoneal carcinomatosis in 3 and undetermined abdominal mass in 3.
Results:
Surgical findings suggested pseudomyxoma peritonei in 8 patients and peritoneal carcinomatosis in 3. Appendicectomy was performed in 9 patients, and in 3 of them bilateral anexectomy was also performed. One patient underwent ileocaecal resection and another a right hemicolectomy. In all the cases, mucinous material was eliminated as much as possible. Pathology revealed mucinous cystoadenoma in 6 cases, mucinous cystoadenocarcinoma in 3 and epithelial hyperplasia in 2 patients. Median survival was 54 months, with a 5- year survival rate of 40%. The last case we treated was sent to a reference centre for the treatment of pseudomyxoma peritonei.
Conclusions:
There is no consensus on the best treatment for pseudomyxoma peritonei. We recommend avoiding incomplete surgical resections in non-reference centres and submitting patients to a reference centre to undergo adequate treatment.
Insights
Pseudomyxoma peritonei, a rare condition of gelatinous abdominal material, was studied in 11 patients. Optimal treatment requires specialized centers to avoid incomplete resections and improve survival rates.
Area of Science:
- Gastroenterology
- Surgical Oncology
- Pathology
Background:
- Pseudomyxoma peritonei is a rare condition characterized by gelatinous abdominal collections and mucinous peritoneal implants.
- It typically arises secondary to a ruptured mucinous tumor, often of ovarian or appendiceal origin.
Purpose of the Study:
- To review the clinical experience with pseudomyxoma peritonei secondary to appendiceal neoplasms.
- To analyze diagnostic methods, surgical interventions, and patient outcomes.
- To provide recommendations for optimal patient management.
Main Methods:
- Retrospective analysis of 11 cases of pseudomyxoma peritonei secondary to appendiceal epithelial neoplasms over 27 years.
- Review of clinical manifestations, imaging findings (CT scan), surgical procedures, and pathological results.
- Survival data analysis, including median survival and 5-year survival rate.
Main Results:
- The mean patient age was 68 years, with common symptoms including abdominal distension and right lower quadrant pain.
- Appendiceal neoplasms were the origin in all cases, with pathology revealing mucinous cystoadenoma, mucinous cystoadenocarcinoma, and epithelial hyperplasia.
- Median survival was 54 months, with a 5-year survival rate of 40%.
Conclusions:
- There is no established consensus on the optimal treatment for pseudomyxoma peritonei.
- Incomplete surgical resections, particularly in non-reference centers, should be avoided.
- Patients should be referred to specialized reference centers for appropriate management and improved outcomes.
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