Neuroendocrine Carcinomas of the Gastroenteropancreatic System: A Comprehensive Review

Diagnostics

Emma Elizabeth Ilett¹  Seppo W. Langer¹  Ingrid Holst Olsen¹  Birgitte Federspiel¹  Andreas Kjær¹  Ulrich Knigge¹ 

[1] Neuroendocrine Tumor Centre of Excellence, Rigshospitalet, University of Copenhagen, 2100 Copenhagen, Denmark; E-Mails:

关键词: neuroendocrine carcinomas;    neuroendocrine tumours;    neuroendocrine neoplasms;    small cell carcinomas;    large cell carcinomas;    Ki-67 index;    oesophagus;    stomach;    pancreas;    colo-rectal;   

DOI  :  10.3390/diagnostics5020119

来源: mdpi

【 摘 要 】

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.

【 授权许可】

CC BY   
© 2015 by the authors; licensee MDPI, Basel, Switzerland.