Early results of surgery in 123 patients with pseudomyxoma peritonei from a perforated appendiceal neoplasm.
OBJECTIVE
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.
Murphy
Elizabeth M
EM
Pseudomyxoma Peritonei Centre, Colorectal Research Unit, North Hampshire Hospital, Basingstoke, Hampshire, United Kingdom.
Sexton
Rosemary
R
Moran
Brendan J
BJ
D000328:Adult; D000368:Aged; D001063:Appendiceal Neoplasms / Q000188:drug therapy / Q000473:pathology* / Q000601:surgery; D014408:Biomarkers, Tumor / Q000032:analysis; D003131:Combined Modality Therapy; D005260:Female; D006801:Humans; D008297:Male; D008875:Middle Aged; D010534:Peritoneal Neoplasms / Q000188:drug therapy / Q000473:pathology* / Q000601:surgery; D011183:Postoperative Complications; D011553:Pseudomyxoma Peritonei / Q000188:drug therapy / Q000473:pathology* / Q000601:surgery; D012421:Rupture
D014408:Biomarkers, Tumor