Purpose: Epithelial appendiceal tumors are uncommon but can present as an emergency simulating appendicitis, or unexpectedly at laparotomy, laparoscopy, or on cross-sectional imaging. Occult rupture with features of pseudomyxoma peritonei may be encountered. We report the operative findings, pathologic assessment, and early outcomes in 123 consecutive patients with a perforated appendiceal neoplasm presenting as pseudomyxoma peritonei.
Methods: From March 1994 to March 2004, 292 patients were referred to a peritoneal malignancy surgical treatment center. Complete tumor removal (cytoreduction) was attempted in selected patients and, if achieved, surgery was combined with intraoperative, intraperitoneal mitomycin C (10 mg/m(2)).
Results: In total, 123 patients (52 males; 41 percent) underwent laparotomy for a perforated appendiceal malignancy presenting as pseudomyxoma peritonei. The median age was 52 (range 30-77) years. Complete cytoreduction was achieved in 83 of 123 patients (67 percent), major palliative resection in 34 patients (28 percent), and 6 patients (5 percent) were inoperable. Postoperative mortality was 6 of 123 patients (5 percent). Kaplan-Meier analysis of the 83 patients who had complete tumor removal predicted 75 percent disease-free survival at five years.
Conclusions: A perforated appendiceal epithelial tumor most frequently presents as pseudomyxoma peritonei. This treatment strategy, involving surgery and intraperitoneal chemotherapy, can result in good outcomes in this rare and otherwise fatal disease.