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Unusual origins of Pseudomyxoma peritonei
E de Bree1, A Witkamp, M Van De Vijver
1Department of Surgical Oncology, The Netherlands Cancer Institute, Amsterdam, The Netherlands. ebree@nki.nl
Abstract:
Pseudomyxoma peritonei is a rare neoplastic condition in that gelatinous intraperitoneal fluid collections and mucinous implants on the peritoneal surfaces and omentum are found. The pathological origin is usually an adenoma or well-differentiated adenocarcinoma of the appendix. A smaller number of cases arises from ovarian tumors. We report two unusual cases of pseudomyxoma peritonei. As a child, the first patient underwent several surgical procedures of the large bowel to relieve the consequences of Hirschsprung disease that were complicated by recurrent enteric fistulae. Colonic epithelial cells, with neoplastic changes due to chronic inflammation in the presence of enteric fistulae, were probably dislocated during these episodes, causing pseudomyxoma peritonei. In the second patient, pseudomyxoma was caused by intraperitoneal seeding of a mucinous urachal adenocarcinoma. We hypothesize that seeding of mucus producing epithelial cells into the abdominal cavity may result in this rare entity called pseudomyxoma peritonei, regardless of the source.
Insights
Pseudomyxoma peritonei, a rare condition with gelatinous fluid and mucinous implants, can arise from unusual sources. This study highlights two cases, one linked to Hirschsprung disease complications and another to urachal adenocarcinoma, emphasizing cell seeding as a potential cause.
Area of Science:
- Oncology
- Gastroenterology
- Pathology
Background:
- Pseudomyxoma peritonei is characterized by gelatinous ascites and peritoneal mucinous implants.
- It typically originates from appendiceal or ovarian mucinous neoplasms.
- Understanding its diverse origins is crucial for diagnosis and management.
Observation:
- Two unusual cases of pseudomyxoma peritonei are presented.
- Case 1 involved a patient with a history of Hirschsprung disease and enteric fistulae.
- Case 2 involved a mucinous urachal adenocarcinoma.
Findings:
- The first case suggests colonic epithelial cell dislocation due to chronic inflammation and enteric fistulae as a potential cause.
- The second case demonstrates intraperitoneal seeding of a mucinous urachal adenocarcinoma.
- Both cases support the hypothesis of mucus-producing epithelial cell seeding leading to pseudomyxoma peritonei.
Implications:
- This broadens the understanding of pseudomyxoma peritonei etiology beyond common appendiceal and ovarian sources.
- It underscores the importance of considering rare origins in clinical diagnosis.
- Further research into epithelial cell seeding mechanisms in pseudomyxoma peritonei is warranted.