Appendiceal neuroendocrine tumors: Recent insights and clinical implications

New insights emerged last decade that enriched our knowledge regarding the biological behavior of appendiceal neuroendocrine tumors (NETs), which range from totally benign tumors less than 1cm to goblet cell carcinomas which behave similarly to colorectal adenocarcinoma. The clinical implication of that knowledge reflected to surgical strategies which also vary from simple appendicectomy to radical abdominal procedures based on specific clinical and histological characteristics. Since the diagnosis is usually established post-appendicectomy, current recommendations focus on the early detection of: (1) the subgroup of patients who require further therapy; (2) the recurrence based on the chromogranin a plasma levels; and (3) other malignancies which are commonly developed in patients with appendiceal NETs.