Primary adenocarcinoma of the appendix: an update

Adenocarcinoid arising in the vermiform appendix is a rarity. The diagnosis of appendiceal adenocarcinoma has never been made preoperatively and is frequently an incidental finding at the time of operation for unrelated conditions. Because of the rarity of the disease, its natural history is poorly understood. Benefits from adjuvant radiation, chemotherapy, or a combination have not been reported. Most studies emphasize survival outcome based on surgical technique. There are few data and, consequently, much debate on the appropriate management of these lesions. The aim of this article is to review this entity and to assess and report new data, through a study of the literature.