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CC BY-NC-ND 4.0 · Indian J Med Paediatr Oncol 2021; 42(05): 470-478
DOI: 10.1055/s-0041-1732833
How I Treat

How I Treat Neuroendocrine Tumors

Aasems Jacob
1   Division of Medical Oncology, University of Kentucky, Lexington, Kentucky, United States
,
Gaby Gabriel
2   Department of Radiology, Division of Interventional Radiology, University of Kentucky, Lexington, Kentucky, United States
3   Division of Medical Oncology, Markey Cancer Center, University of Kentucky, Lexington, Kentucky, United States
,
Robert A. Ramirez
4   Department of Hematology and Oncology, Ochsner Health New Orleans, New Orleans, Louisiana, United States
,
Yi-Zarn Wang
5   Department of Surgery, Louisiana State University Health Science Center, New Orleans, Louisiana, United States
,
Lowell Anthony
1   Division of Medical Oncology, University of Kentucky, Lexington, Kentucky, United States
3   Division of Medical Oncology, Markey Cancer Center, University of Kentucky, Lexington, Kentucky, United States
,
Aman Chauhan
1   Division of Medical Oncology, University of Kentucky, Lexington, Kentucky, United States
3   Division of Medical Oncology, Markey Cancer Center, University of Kentucky, Lexington, Kentucky, United States
› Author Affiliations Financial Disclosures A.C. receives research support from BMS, Clovis Oncology, EMD Serono, Nanopharmaceuticals, Tersera, ECS Progastrin. Advisor: Novartis, Ipsen, TerSera, Lexicon, Seneca Therapeutics, and ECS Progastrin. R.A.R. serves as a speaker for Merck & Co., Inc., Advanced Accelerator Applications, Genentech, Inc., AstraZeneca, and Ipsen Biopharmaceuticals, Inc. Consultant for Curium Pharma, Advanced Accelerator Applications, AstraZeneca, Ipsen Biopharmaceuticals and Novartis Pharmaceuticals, Corp. Research funding from Aadi Biosciences, and Merck & Co., Inc. Other coauthors do not have relevant disclosures.
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Introduction

Neuroendocrine tumors (NETs) originate from diffuse neuroendocrine cell system and can develop in many organs. Gastroenteropancreatic (GEP) NETs account for approximately 70%, followed by bronchopulmonary and thymic NETs.[1] The World Health Organization (WHO) classification divides GEP NETs into well-differentiated NETs and poorly differentiated neuroendocrine carcinoma (NEC). Well-differentiated NETs can be grade 1 (G1; mitotic count <2 per 10 HPF - high power field, Ki-67 < 3%), G2 (mitotic count: 2–20, Ki-67: 3–20%) tumors, and G3 (mitotic count >20, Ki-67 > 20%).[2] Poorly differentiated NECs are always G3 tumors with >20 mitotic count and Ki-67 index >20% and include small- and large-cell NECs.[2] A total of 10 to 13% of NETs do not have a primary site identified at the time of diagnosis and are called NETs of unknown primary.[1] [3] NETs can also be differentiated based on the secretion of vasoactive amines and hormones into functional (30%) and nonfunctional NETs (70%).[1] This article focuses on the management of well-differentiated NETs with attention to systemic therapy. Factors influencing initial medical decision-making in NET management include functional status, stage, and grade, burden of metastatic disease, and symptoms at presentation.



Publication History

Article published online:
23 September 2021

© 2021. Indian Society of Medical and Paediatric Oncology. This is an open access article published by Thieme under the terms of the Creative Commons Attribution-NonDerivative-NonCommercial License, permitting copying and reproduction so long as the original work is given appropriate credit. Contents may not be used for commercial purposes, or adapted, remixed, transformed or built upon. (https://creativecommons.org/licenses/by-nc-nd/4.0/)

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