K I Alexandraki et al. Diagnosis and treatment 23:1 R27–R41 Review of appendiceal NEN Appendiceal neuroendocrine neoplasms: diagnosis and management Krystallenia I Alexandraki1, Gregory A Kaltsas1, Simona Grozinsky-Glasberg2, Eleftherios Chatzellis1 and Ashley B Grossman3 Correspondence 1Department of Pathophysiology, National University of Athens, Greece should be addressed 2Neuroendocrine Tumor Unit, Endocrinology and Metabolism Service, Department of Medicine, to K Alexandraki Hadassah-Hebrew University Hospital, Jerusalem, Israel Email 3Oxford Centre for Diabetes, Endocrinology and Metabolism, Churchill Hospital, University of Oxford, Oxford, UK
[email protected] Abstract Gastrointestinal neuroendocrine neoplasms (GI-NENs) are increasingly being recognised, Key Words while appendiceal NENs (aNENs) currently constitute the third most common GI-NEN. " neuroendocrine Appendiceal NENs are generally considered to follow an indolent course with the majority " neoplasm being localised at diagnosis. Thus, the initial surgical approach is not that of a planned " appendiceal oncological resection. Due to the localised nature of the disease in the majority of cases, " appendicectomy subsequent biochemical and radiological assessment are not routinely recommended. " right hemicolectomy Histopathological criteria (size, mesoappendiceal invasion, Ki-67 proliferation index, neuro- " carcinoid and angio-invasion) are mainly used to identify those patients who are also candidates for a Endocrine-Related Cancer right hemicolectomy. Goblet cell carcinoids are a distinct entity and should be treated as adenocarcinomas. Despite the absence of any substantial prospective data regarding optimal management and follow-up, recent consensus statements and guidelines have been published. The purpose of this review is to overview the published studies on the diagnosis and management of appendiceal NENs and to suggest a possible management protocol.