Pseudomyxoma Peritonie Is A Rare Clinically Challenging Entity: Review Of Three Cases

Abstract

Background: Pseudomyxoma Peritonei is a rare disease. It is identified operatively by gelatinous ascites accompanied by mucinous material in the peritoneum, usually arising from appendix and ovary. Other sites from where it may arise are colon, gallbladder, pancreas or stomach. The clinical presentation is inconsistent and preoperative diagnosis is often difficult. The focus of this study is to consider the clinical, histopathological aspects and survival of pseudomyxoma-peritonie in a tertiary care hospital of Karachi. Methods: A single center Ziauddin University and hospital, histopathology database from 2006-2016 was looked up retrospectively for the incidence of pseudomyxoma-peritonie on biopsy and reviewed patient’s record. All cases operated on the basis of clinical presentation and radiological findings. Diagnosis made incidentally on histopathology, originated from three different sites. Results: Total 1388 appendectomies were performed, 749(53.96%) were male, and 639(46.03%) female. Carcinoid tumor was present in 11(0.79%) and 3(0.21%) were of pseudomyxoma-peritonie variety. In the later 3, two were males and one was a female with ages in 42, 85, 52 years respectively. All three-patients came to the emergency with variable presentation. The preoperative diagnosis was of a perforated appendix, large mesenteric cyst and in the third-case a large cystic-cum-solid abdominal mass. Histopathology confirmed pseudomyxoma-peritonie. The primary sites diagnosed were appendix, pancreas and ovary. Conclusion: Pseudomyxoma-peritonie is a challenging task to diagnose clinically. On the basis of case series, this intra-peritoneal tumor has varied presentation. Patients usually present in late stage of disease with mucinous tumor cells implanted in the peritoneal mesothelium and in clinically apparent ascites. Key Words: Pseudomyxoma-peritonie, Mucinous tumor, Appendiceal tumor, Peritoneal carcinomatosis, Rare entity