Abstract
An abnormality was noted on abdominal computed tomography (CT) of a 70-year-old man, and he was referred to our department. Abdominal contrast-enhanced CT showed dilation of the appendix and wall thickening of the cecum. Colonoscopy showed a submucosal mass with smooth margins in the cecum, and a diagnosis of appendiceal tumor was made. Because a malignant appendiceal tumor was possible, ileocecal resection with D2 lymph node dissection was performed. The pathological diagnosis was appendiceal GIST with mucinous cystadenoma. Since appendiceal GIST is very rare among GISTs, this case is reported along with a review of the relevant literature.