The spectrum of neuroendocrine tumors: Histologic classification, unique features and areas of overlap Journal Article


Authors: Klimstra, D. S.; Beltran, H.; Lilenbaum, R.; Bergsland, E.
Editor: Dizon, D. S.
Article Title: The spectrum of neuroendocrine tumors: Histologic classification, unique features and areas of overlap
Abstract: Neuroendocrine neoplasms are diverse in terms of sites of origin, functional status, and degrees of aggressiveness. This review will introduce some of the common features of neuroendocrine neoplasms and will explore the differences in pathology, classification, biology, and clinical management between tumors of different anatomic sites, specifically, the lung, pancreas, and prostate. Despite sharing neuroendocrine differentiation and histologic evidence of the neuroendocrine phenotype in most organs, well-differentiated neuroendocrine tumors (WD-NETs) and poorly differentiated neuroendocrine carcinomas (PD-NECs) are two very different families of neoplasms. WD-NETs (grade 1 and 2) are relatively indolent (with a natural history that can evolve over many years or decades), closely resemble non-neoplastic neuroendocrine cells, and demonstrate production of neurosecretory proteins, such as chromogranin A. They arise in the lungs and throughout the gastrointestinal tract and pancreas, but WD-NETs of the prostate gland are uncommon. Surgical resection is the mainstay of therapy, but treatment of unresectable disease depends on the site of origin. In contrast, PD-NECs (grade 3, small cell or large cell) of all sites often demonstrate alterations in P53 and Rb, exhibit an aggressive clinical course, and are treated with platinum-based chemotherapy. Only WD-NETs arise in patients with inherited neuroendocrine neoplasia syndromes (e.g., multiple endocrine neoplasia type 1), and some common genetic alterations are site-specific (e.g., TMPRSS2-ERG gene rearrangement in PD-NECs arising in the prostate gland). Advances in our understanding of the molecular basis of NETs should lead to new diagnostic and therapeutic strategies and is an area of active investigation.
Keywords: pancreatic neoplasms; classification; lung neoplasms; pathology; prostatic neoplasms; neuroendocrine tumors; humans; human; male
Journal Title: American Society of Clinical Oncology Educational Book
ISSN: 1548-8756
Publisher: American Society of Clinical Oncology  
Date Published: 2015-01-01
Start Page: 92
End Page: 103
Language: English
DOI: 10.14694/EdBook_AM.2015.35.92
PUBMED: 25993147
PROVIDER: scopus
DOI/URL:
Notes: Review -- Export Date: 2 November 2016 -- Source: Scopus
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  1. David S Klimstra
    945 Klimstra