title: Neuroendocrine Carcinomas of the Gastroenteropancreatic System: A Comprehensive Review.
abstract: To date, empirical literature has generally been considered lacking in relation to neuroendocrine carcinomas (NECs), the highly malignant subgroup of neuroendocrine neoplasms. NECs are often found in the lungs or the gastroenteropancreatic (GEP) system and can be of small or large cell type. Concentrating on GEP-NECs, we can conclude that survival times are poor, with a median of only 4-16 months depending on disease stage and primary site. Further, this aggressive disease appears to be on the rise, with incidence numbers increasing while survival times are stagnant. Treatment strategies concerning surgery are often undecided and second-line chemotherapy is not yet established. After an analysis of over 2600 articles, we can conclude that there is indeed more empirical literature concerning GEP-NECs available than previously assumed. This unique review is based on 333 selected articles and contains detailed information concerning all aspects of GEP-NECs. Namely, the classification, histology, genetic abnormalities, epidemiology, origin, biochemistry, imaging, treatment and survival of GEP-NECs are described. Also, organ-specific summaries with more detail in relation to disease presentation, diagnosis, treatment and survival are presented. Finally, key points are discussed with directions for future research priorities.
authors: Neuroendocrine Tumor Centre of Excellence, Rigshospitalet, University of Copenhagen, 2100 Copenhagen, Denmark. ilett@sund.ku.dk.|Department of Gastrointestinal Surgery C, Rigshospitalet, University of Copenhagen, 2100 Copenhagen, Denmark. ilett@sund.ku.dk.
authors: Emma Elizabeth
authors: EE
authors: Ilett
authors: Neuroendocrine Tumor Centre of Excellence, Rigshospitalet, University of Copenhagen, 2100 Copenhagen, Denmark. seppo.langer@regionh.dk.|Department of Oncology, Rigshospitalet, University of Copenhagen, 2100 Copenhagen, Denmark. seppo.langer@regionh.dk.
authors: Seppo W
authors: SW
authors: Langer
authors: Neuroendocrine Tumor Centre of Excellence, Rigshospitalet, University of Copenhagen, 2100 Copenhagen, Denmark.|Department of Gastrointestinal Surgery C, Rigshospitalet, University of Copenhagen, 2100 Copenhagen, Denmark.|Cluster for Molecular Imaging, Faculty of Health Sciences, University of Copenhagen, 2100 Copenhagen, Denmark.
authors: Ingrid Holst
authors: IH
authors: Olsen
authors: Neuroendocrine Tumor Centre of Excellence, Rigshospitalet, University of Copenhagen, 2100 Copenhagen, Denmark. birgitte.federspiel@regionh.dk.|Department of Pathology, Rigshospitalet, University of Copenhagen, 2100 Copenhagen, Denmark. birgitte.federspiel@regionh.dk.
authors: Birgitte
authors: B
authors: Federspiel
authors: Neuroendocrine Tumor Centre of Excellence, Rigshospitalet, University of Copenhagen, 2100 Copenhagen, Denmark. andreas.kjaer@regionh.dk.|Cluster for Molecular Imaging, Faculty of Health Sciences, University of Copenhagen, 2100 Copenhagen, Denmark. andreas.kjaer@regionh.dk.|Department of Clinical Physiology, Nuclear Medicine and PET, Rigshospitalet, University of Copenhagen, 2100 Copenhagen, Denmark. andreas.kjaer@regionh.dk.
authors: Andreas
authors: A
authors: Kjær
authors: Neuroendocrine Tumor Centre of Excellence, Rigshospitalet, University of Copenhagen, 2100 Copenhagen, Denmark. ulrich.peter.knigge@regionh.dk.|Department of Gastrointestinal Surgery C, Rigshospitalet, University of Copenhagen, 2100 Copenhagen, Denmark. ulrich.peter.knigge@regionh.dk.|Cluster for Molecular Imaging, Faculty of Health Sciences, University of Copenhagen, 2100 Copenhagen, Denmark. ulrich.peter.knigge@regionh.dk.|Deparment of Endocrinology, Rigshospitalet, University of Copenhagen, 2100 Copenhagen, Denmark. ulrich.peter.knigge@regionh.dk.
authors: Ulrich
authors: U
authors: Knigge
keywords: Ki-67 index; colo-rectal; large cell carcinomas; neuroendocrine carcinomas; neuroendocrine neoplasms; neuroendocrine tumours; oesophagus; pancreas; small cell carcinomas; stomach