Digestive System Mixed Neuroendocrine-Non-Neuroendocrine Neoplasms

• Published in: Neuroendocrinology Vol. 105; no. 4; p. 412
• Main Authors: de Mestier, Louis, Cros, Jérôme, Neuzillet, Cindy, Hentic, Olivia, Egal, Axel, Muller, Nelly, Bouché, Olivier, Cadiot, Guillaume, Ruszniewski, Philippe, Couvelard, Anne, Hammel, Pascal
• Format: Journal Article
• Language: English
• Published: Switzerland 01.01.2017
• Subjects: B-Cell CLL-Lymphoma 10 Protein - metabolism; Carcinoma - classification; Carcinoma - diagnosis; Carcinoma - genetics; Carcinoma - pathology; Chromogranin A - metabolism; Digestive System - metabolism; Digestive System - pathology; Disease Management; Humans; Mutation - genetics; Neoplasm Proteins - metabolism; Neuroendocrine Tumors - classification; Neuroendocrine Tumors - diagnosis; Neuroendocrine Tumors - pathology; Adenocarcinoma; Chemotherapy; Neuroendocrine tumours; Prognosis; Treatment; Mixed tumours; Epidemiology
• ISSN: 1423-0194
• DOI: 10.1159/000475527

Mixed neuroendocrine-non-neuroendocrine neoplasms (MiNEN) are a heterogeneous subgroup of rare neoplasms that represent about a third of all poorly differentiated neuroendocrine carcinomas (PDNEC). MiNEN combine a neuroendocrine component, usually a PDNEC, and a non-neuroendocrine component, generally an adenocarcinoma, both accounting for at least 30% of the neoplasm. MiNEN are classified as high-, intermediate-, or low-grade malignancies depending on the metastatic potential of the tumour components. High-grade malignant component should be considered even if it represents <30% of the tumour. The prognosis of MiNEN is generally intermediate between those of the two "pure" components composing it. The aim of this comprehensive review of the literature is to suggest a standardized management of MiNEN. An increasing body of evidence suggests that PDNEC components share molecular abnormalities with their adenocarcinoma counterparts, but also display additional alterations. This advocates for a common origin, and that the presence of a PDNEC component in an adenocarcinoma could indicate a turning point in carcinogenesis.