Early Results of Surgery in 123 Patients With Pseudomyxoma Peritonei From a Perforated Appendiceal Neoplasm

Dis Colon Rectum. 2007 Jan;50(1):37-42. doi: 10.1007/s10350-006-0741-9.


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.

MeSH terms

  • Adult
  • Aged
  • Appendiceal Neoplasms / drug therapy
  • Appendiceal Neoplasms / pathology*
  • Appendiceal Neoplasms / surgery
  • Biomarkers, Tumor / analysis
  • Combined Modality Therapy
  • Female
  • Humans
  • Male
  • Middle Aged
  • Peritoneal Neoplasms / drug therapy
  • Peritoneal Neoplasms / pathology*
  • Peritoneal Neoplasms / surgery
  • Postoperative Complications
  • Pseudomyxoma Peritonei / drug therapy
  • Pseudomyxoma Peritonei / pathology*
  • Pseudomyxoma Peritonei / surgery
  • Rupture


  • Biomarkers, Tumor