A Case of Pseudomyxoma Retroperitonei

Abstract

Question: An 80-year-old woman was admitted for the detailed examination of a lower abdominal mass. She had neither pain nor digestive symptoms. A fist-sized, hard, smooth-surfaced mass was palpated at the right lower quadrant. The laboratory data showed moderate anemia and slightly increased cancer antigen 19-9. The extrinsic and smooth compression of the terminal ileum was the only finding by the contrast enema. Abdominal computed tomography showed a well-defined mass with cystic components in the right iliac fossa with its thick capsule enhanced by the contrast media and the rod-shaped material was identified in the cystic tumor (Figure A). It pushed up the cecum and caused hydronephrosis by compressing the right ureter. Sagittal section magnetic resonance imaging (MRI) showed a small ring in the center of the mass (Figure B). Colonoscopy showed a benign ulcer in the cecum. What is the diagnosis for this cystic tumor?View Large Image Figure ViewerDownload Hi-res image Download (PPT) Look on page 372 for the answer and see the Gastroenterology website (www.gastrojournal.org) for more information on submitting your favorite image to Clinical Challenges and Images in GI. At laparotomy, the peritoneal cavity was free of fluid and in the right lower quadrant was retroperitoneally bulging and adhesively covered by colon and right ovary. We successfully resected the tumor along with ileum, cecum, both ovaries, and uterus without damaging the mass itself. Macroscopically, it was a ball of about 10 cm in diameter containing gelatinous substance like pseudomyxoma peritonei. Thus, we diagnosed it as pseudomyxoma retroperitonei. Histopathologically, moderately differentiated and mucinous adenocarcinoma occupied the inner wall of the cavity (Figure C). The rod-like body at the core of the mass, which was clearly shown in MRI imaging, that was covered with cancer cells both inside and outside turned out to be the appendix (Figure D). Therefore, we speculate that appendiceal cancer perforated into the retroperitoneum and gradually formed the cystic mass with thick fibrous wall.View Large Image Figure ViewerDownload Hi-res image Download (PPT) Pseudomyxoma peritonei is a rare and intractable disease that fills the abdominal cavity with gelatinous material. Originating from appendix or ovary, the mucus-producing neoplastic cells usually spread all over the peritoneal surface. They keep producing the jelly that conglutinate or compress bowels, which sometimes causes intestinal obstruction and fatal malnutrition. Complete remission is difficult to achieve without extensive peritonectomy, including the disseminated neoplastic deposits. Retroperitoneally confined pseudomyxoma as in this case is quite rare. However, a good prognosis can be expected with its intact removal.1Solkar M.H. Akhtar N.M. Khan Z. Parker M.C. Pseudomyxoma extraperitonei occurring 35 years after appendicectomy: a case report and review of literature.World J Surg Oncol. 2004; 2: 19Crossref PubMed Scopus (19) Google Scholar