Abstract
Simple SummaryNeuroendocrine neoplasms are divided into two groups: well-differentiated neuroendocrine tumors and poorly differentiated neuroendocrine carcinomas. The progress in diagnostic methods, including pathology optimization and imaging, might be one of the reasons for the increasing incidence of gastroenteropancreatic neuroendocrine neoplasms; however, the remaining biological factors are undetermined. Rapid advances in molecular diagnostic and treatment strategies in recent years have significantly contributed to personalized management for patients with these rare neoplasms. This review aimed to provide an update on the epidemiology, diagnosis, and biomarkers in gastroenteropancreatic neuroendocrine neoplasms.Gastroenteropancreatic neuroendocrine neoplasms (GEP-NENs) are a heterogeneous group of malignancies that originate from the diffuse neuroendocrine cell system of the pancreas and gastrointestinal tract and have increasingly increased in number over the decades. GEP-NENs are roughly classified into well-differentiated neuroendocrine tumors and poorly differentiated neuroendocrine carcinomas; it is essential to understand the pathological classification according to the mitotic count and Ki67 proliferation index. In addition, with the advent of molecular-targeted drugs and somatostatin analogs and advances in endoscopic and surgical treatments, the multidisciplinary treatment of GEP-NENs has made great progress. In the management of GEP-NENs, accurate diagnosis is key for the proper selection among these diversified treatment methods. The evaluation of hormone-producing ability, diagnostic imaging, and histological diagnosis is central. Advances in the study of the genetic landscape have led to deeper understanding of tumor biology; it has also become possible to identify druggable mutations and predict therapeutic effects. Liquid biopsy, based on blood mRNA expression for GEP-NENs, has been developed, and is useful not only for early detection but also for assessing minimal residual disease after surgery and prediction of therapeutic effects. This review outlines the updates and future prospects of the epidemiology, diagnosis, and management of GEP-NENs.
Highlights
Neuroendocrine neoplasms (NENs) are a group of epithelial tumors with morphological and immunohistochemical features of neuroendocrine differentiation [1]
This review focuses on the updated findings of the epidemiology, diagnosis, genetic data, and future perspectives of gastroenteropancreatic (GEP)-NENs
There has been a rapid increase in the number of clinically identified GEP-NENs in the last few decades
Summary
Neuroendocrine neoplasms (NENs) are a group of epithelial tumors with morphological and immunohistochemical features of neuroendocrine differentiation [1]. All NENs share similar configurations and specific neuroendocrine expressions, they behave very differently in relation to the site of origin, histological grade, clinical stage, and hormone production [3,4]. The clinical presentation and prognosis of NENs are diverse; various diagnostic and therapeutic approaches have been attempted to date. Recent advances in genomic and epigenetic sciences have provided significant benefits in oncology [8,9,10], whereas the evidence is insufficient for NENs. . NENs are considered rare, their incidence has been increasing globally, which in turn has received more attention from clinicians and researchers in recent years. This review focuses on the updated findings of the epidemiology, diagnosis, genetic data, and future perspectives of gastroenteropancreatic (GEP)-NENs
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