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.

ENETS Consensus Guidelines Update for the Management of Distant Metastatic Disease of Intestinal, Pancreatic, Bronchial Neuroendocrine Neoplasms (NEN) and NEN of Unknown Primary Site

“…Targeted drugs in combination with chemotherapy are under evaluation in clinical trials on G3 NEN. Further details on the management of G3 NEN, including recommendations for different primary tumor sites, are summarized in a recently published comprehensive review on G3 NEC and are provided in a separate consensus paper [82,83]. …”

Interleukins 17 and 23 in patients with gastric neoplasms

“…Other less frequently occurring types of neoplasms, which may have milder and less debilitating clinical outcomes, may also develop within the gastric tissue. These include gastrointestinal stromal tumors (GISTs), neuroendocrine neoplasms (NENs), and/or diverse types of gastric lymphomas456789. However, even though significant effort has been directed toward elucidating the pathogenesis of various types of gastric neoplasms, the exact mechanisms and factors responsible for the development and/or progression of these tumors in humans remain unknown.…”

Establishment and Characterization of a Novel Cell Line Derived from a Small Cell Neuroendocrine Carcinoma of the Anal Canal

“…To date, therapy approaches for GEP-NECs are largely adopted from treatment strategies of small cell lung cancer [19][20][21][22]. Median survival rates for GEP-NEC reported in recent studies range from 4 to 16 months [7,9,10,17,[21][22][23][24]. In this context, it becomes evident that there is an urgent need for novel therapeutic strategies specifically developed for GEP-NEC.…”