Nothing But NET: A Review of Neuroendocrine Tumors and Carcinomas

This review covers the diverse topic of neuroendocrine neoplasms (NENs), a relatively rare and heterogeneous tumor type, comprising ~2% of all malignancies, with a prevalence of <200,000 in the United States, which makes it an orphan disease (Basu et al., 2010).1 For functional purposes, NENs are...

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Veröffentlicht in:Neoplasia (New York, N.Y.) Jg. 19; H. 12; S. 991 - 1002
Hauptverfasser: Oronsky, Bryan, Ma, Patrick C., Morgensztern, Daniel, Carter, Corey A.
Format: Journal Article
Sprache:Englisch
Veröffentlicht: United States Elsevier Inc 01.12.2017
Elsevier Limited
Neoplasia Press
Elsevier
Schlagworte:
Algorithms
Animals
Biomarkers, Tumor
Carcinoma
Carcinoma, Neuroendocrine - diagnosis
Carcinoma, Neuroendocrine - epidemiology
Carcinoma, Neuroendocrine - etiology
Carcinoma, Neuroendocrine - therapy
Disease Management
Disease Susceptibility
Humans
Neoplasm Grading
Neoplasm Staging
Neuroendocrine tumors
Neuroendocrine Tumors - diagnosis
Neuroendocrine Tumors - epidemiology
Neuroendocrine Tumors - etiology
Neuroendocrine Tumors - therapy
Review article
Reviews
Tumors
ISSN:1476-5586, 1522-8002, 1476-5586, 1522-8002
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Beschreibung
Zusammenfassung:This review covers the diverse topic of neuroendocrine neoplasms (NENs), a relatively rare and heterogeneous tumor type, comprising ~2% of all malignancies, with a prevalence of <200,000 in the United States, which makes it an orphan disease (Basu et al., 2010).1 For functional purposes, NENs are divided into two groups on the basis of clinical behavior, histology, and proliferation rate: well differentiated (low grade to intermediate grade) neuroendocrine tumors and poorly differentiated (high grade) neuroendocrine carcinoma (Bosman et al., 2010)2; this histological categorization/dichotomization is highly clinically relevant with respect to impact on treatment and prognosis even though it is not absolute since a subset of tumors with a low-grade appearance behaves similarly to high-grade lesions. Given the relative dearth of evidenced-based literature about this orphan disease as a whole (Modlin et al., 2008),3 since the focus of most articles is on particular anatomic subtypes of NENs (i.e., gastroenteropancreatic or pulmonary), the purpose of this review is to summarize the presentation, pathophysiology, staging, current standard of care treatments, and active areas of current research.
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ISSN:1476-5586
1522-8002
1476-5586
1522-8002
DOI:10.1016/j.neo.2017.09.002