Purpose of review: To synthesize the current knowledge and guidelines on the management of appendiceal neuroendocrine neoplasms (ANENs).
Recent findings: Most recent guidelines are essentially based on heterogeneous retrospective series. With the advent of a more precise classification of neuroendocrine neoplasms, this heterogeneity is rightly criticized and many 'grey areas' are now debated in expert literature. The only way to solve these issues is through the conduct of large prospective multicentre studies, but this seems somewhat utopian, given the rarity of this disease.
Summary: ANENs are rare tumours with a favourable prognosis, and mainly diagnosed in young patients. They are predominantly localized, and diagnosed incidentally on appendectomy. This procedure is curative for the vast majority of patients but ANENs can relapse even a significant time after the first diagnosis. Identifying the risks for recurrence is challenging, with some factors thought to be predictive of nodal involvement. The presence of one or more of these factors justifies an oncological radicalization of the surgical procedure (right hemicolectomy with lymphadenectomy). However, the beneficial impact of this surgical procedure on survival is still unproven.