Appendiceal Goblet Cell Adenocarcinoma Case Report and Review of the Literature

Acute appendicitis is a common presentation to the emergency department. The common pathogenesis thereof relates to obstruction of the appendiceal lumen by an appendicolith, which leads to an increase in intraluminal and intramural pressure. This is followed by distension of the appendix, subsequent small vessel occlusion and lymphatic stasis, and appendiceal wall ischemia and necrosis, eventually leading to rupture if not treated. Occasionally tumors at the base of the appendix can lead to appendicitis via the same process as an appendicolith. Goblet cell adenocarcinoma (alternatively named goblet cell carcinoid) is amongst the most rare appendiceal tumors, with a reported incidence rate of 0.05 cases per 100,000 population per year in the United States. These tumors contain features of both neuroendocrine tumors as well as adenocarcinomas, but behave more similarly to adenocarcinomas. Consensus regarding management of these tumors is lacking, likely due to the rarity of the disease. In this paper, we present a case of appendiceal goblet cell adenocarcinoma causing appendicitis and review the literature regarding these rare epithelial tumors.