Background: Previous reports on the surgical management of appendix cancer show high recurrence rates among patients initially presenting with localized disease. This study sought to characterize predictors of outcome among patients treated for stages 1-3 appendix cancer at the authors' institution.
Methods: Patients with nonmetastatic appendix cancer undergoing definitive surgery at a single cancer center from 1994 to 2013 were retrospectively reviewed. Patients with appendiceal adenomas, cystadenomas, or classical carcinoids were excluded from the study. The median follow-up period was 5.2 years (interquartile range 2.9-6.7 years).
Results: The study identified 70 patients, 49 % of whom were women. The median age was 52 years (range 20-84 years). All were explored by an expert surgeon who had treated at least 20 appendiceal cancers. The procedures were appendectomy (n = 2), right hemicolectomy (n = 66), and diagnostic laparoscopy and placement of an intraperitoneal port (n = 2). The final pathology showed that transmural (30 T4, 32 T3, 4 T2, 4 T1) and node-negative disease (80 %) were common. Goblet cell carcinoid (GCC) features were identified in 54 % of the tumors. These were smaller and more likely to present as acute appendicitis than appendiceal adenocarcinoma (AA), but were otherwise similar in clinical presentation and outcome. The presence of lymph node (LN) metastasis was associated with a higher risk of recurrence than of stage 2 appendix cancer (78 vs. 4 % at 5 years; p < 0.0001). A total of 12 patients experienced recurrence (5 GCC, 7 AA): 9 in the peritoneum, 2 in mesenteric LNs, and 1 in the surgical incision.
Conclusion: Stages 1-3 invasive AA and GCC behave similarly in terms of clinical presentation and outcome. Perforated appendix and T4 tumor stage were common but not associated with recurrence. Although uncommon, LN metastasis strongly predicted recurrence.