Mixed neuroendocrine-non-neuroendocrine neoplasms of the digestive system: A mini-review

Abstract

Mixed neuroendocrine-non-neuroendocrine neoplasms (MiNENs) are rare mixed tumors containing both neuroendocrine (NE) and non-NE components. Each component must occupy at least 30% of the tumor volume by definition. Recent molecular evidence suggests MiNENs are clonal neoplasms and potentially harbor targetable mutations similar to conventional carcinomas. There have been multiple changes in the nomenclature and classification of MiNENs which has created some confusion among pathologists on how to integrate the contributions of each component in a MiNEN, an issue which in turn has resulted in confusion in communication with front-line treating oncologists. This mini review summarizes our current understanding of MiNENs and outline diagnosis, prognosis, and management of these neoplasms. The authors emphasize the importance of treating the most aggressive component of the tumor regardless of its percentage volume.

Keywords: Mixed neuroendocrine non-neuroendocrine neoplasms; Mixed adenoneuorendocrine carcinoma; Mixed neuroendocrine neoplasms; Neuroendocrine; Non-neuroendocrine

Core Tip: Mixed neuroendocrine neoplasms have been referred to by a long list of names. The latest term, mixed neuroendocrine-non-neuroendocrine neoplasm (MiNEN), captures a wider spectrum of neoplasms than did previous nomenclature. This mini review summarizes the development of the term MiNEN and reviews current knowledge about the molecular pathogenesis, diagnosis, prognosis, and management of these neoplasms. MiNENs are viewed as clonal neoplasms and their clinical behaviour and management are ultimately determined by the most aggressive component present.