PURPOSE: Adenocarcinoma of the appendix is a rare neoplasm, and contro
versies persist regarding management. The purpose of this study was to
identify prognostic factors and define management strategies for pati
ents with adenocarcinoma of the appendix. METHODS: A retrospective cas
e series was conducted at three medical school teaching hospitals over
a 20-year period from 1972 to 1992. Overall survival was determined b
y the actuarial life table method. Comparisons of prognostic factors w
ere made using exact nonparametric log-rank tests. RESULTS: Thirteen p
atients were diagnosed during the study period. Median age was 62 year
s. There were five males and eight females. The disease was not suspec
ted in any patient preoperatively. Seventy-seven percent of patients h
ad metastatic disease at presentation. Second primary malignancies mer
e found in 15 percent of patients. Thirty-eight percent of female pati
ents had synchronous ovarian lesions. Median survival was 22 months, w
ith an estimated five-year survival of 43 percent (95 percent confiden
ce interval, 22-84 percent). Patients with colonic histology had signi
ficantly worse survival than patients with mucinous histology (P = 0.0
093). Patients with carcinomatosis had a significantly worse survival
than noncarcinomatosis patients (P = 0.0078). Patients who underwent r
ight hemicolectomy had a better prognosis for survival than appendecto
my patients, but the difference was not statistically significant. CON
CLUSIONS: Carcinoma of the appendix is very difficult to diagnose preo
peratively, and most patients are not identified until disease is adva
nced. Good prognostic factors include mucinous histology and the absen
ce of carcinomatosis. Right hemicolectomy appears to be a reasonable o
ption, although its superiority to appendectomy alone has not been. de
finitively proven. High frequency of ovarian metastases in women sugge
sts a role for bilateral oophorectomy. In addition, a complete work-up
of the patient for a synchronous malignancy, especially in the gastro
intestinal tract, should be considered.