Case presentation: A 53-year-old man had noted a very vague sensation of abdominal discomfort for 1 year, which gradually increased over 2 months. He described diffuse abdominal fullness, without a change in weight, appetite, satiety, or abdominal girth. Physical examination was unremarkable. Initial evaluation, including abdominal ultrasound and upper GI endoscopy, was unrevealing. Colonoscopy suggested extrinsic compression of the cecum. Carcinoembryonic antigen level was markedly elevated at 74.8 (reference range 0–3.0 ng/ml). CT scan showed a number of large, soft-tissue masses infiltrating the abdomen and pelvis, and a relatively fixed right lower quadrant mass. A laparotomy was recommended. Operative findings: There were a number of large, mucinous-appearing implants present within the abdominal cavity, consistent with pseudomyxoma peritonei. The serosal surfaces of the spleen, gallbladder, and small bowel, and the diaphragm undersurface all contained several large implants. There were numerous pelvic implants as well. In the right lower quadrant, there was a 29 × 11 × 9 cm mass, which involved the appendix, ileum and right colon. Extensive cytoreductive surgery was performed, removing all bulky visible tumor. Pathology from all areas revealed Grade 2/4 mucinous adenocarcinoma, arising initially from the appendix. Post-operative therapy: Adjuvant therapy with 5-Fluorouracil was administered via two indwelling intraperitoneal catheters, followed by intraperitoneal infusion of chromic P-32 (radioactive phosphate). Discussion: Pseudomyxoma peritonei is an uncommon, often morbid condition in which mucinous adenocarcinoma deposits accumulate in the peritoneal cavity. The etiology is often a primary appendiceal tumor; however, in females ovarian primaries may be implicated. Pseudomyxoma peritonei may be found incidentally at laparotomy, or patients may present with abdominal pain, increased abdominal girth, and palpable abdominal masses. Treatment entails extensive surgical resection, followed by intraperitoneal chemotherapy. Prognosis with extensive disease can be poor, but complete cytoreductive surgery and chemotherapy improves survival. This case highlights the fact that some patients with pseudomyxoma peritonei present with very minimal symptoms, yet demonstrate extensive areas of intraabdominal tumor.
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