Management and Outcomes in Primary Tumors of the Appendix

Primary tumors of the appendix are rare, comprising 1.1% of all appendectomy specimens. Nevertheless, it often presents in an emergent fashion, creating a need for a well-defined management algorithm that will ensure proper acute management. We performed a retrospective review of medical charts from 1982-2007 on all charts with a diagnosis of appendiceal neoplasm. A cohort of 41 patients was diagnosed with a primary appendiceal neoplasm from a total of 8560 appendectomies over the 25-year period. Several tumors were identified: adenocarcinomas (n = 16), carcinoid tumors (n = 15), mucinous cystadenocarcinoma (n = 7), and a combination of adenocarcinoma and goblet cell carcinoid of the appendix (n = 3). Twenty-one patients presented with an acute abdomen. Tumors were discovered intraoperatively in eighteen patients while performing other procedures. At diagnosis, metastatic disease was found in 41.5% of patients (n = 17); average survival ranged from 6 to 21 months based on tumor type. For patients with non-metastatic disease at diagnosis, all survived longer than 2 years and there were no cases of recurrence or post-operative metastasis. We devised an operative strategy dictated by initial presenting characteristics of the tumor. The presence of carcinoma should be suspected and searched for in patients over 40 presenting with acute appendicitis. Intraoperatively any suspicious mass should undergo frozen sectioning as the finding of a malignancy often necessitates a larger or repeat operation. The propensity of these neoplasms for presentation in the guise of acute appendicitis mandates that the surgeon be familiar with the appropriate management algorithm, both in and out of the operating room.