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Korean J Gastroenterol  <  Volume 73(3); 2019 <  Articles

Korean J Gastroenterol 2019; 73(3): 124-131  https://doi.org/10.4166/kjg.2019.73.3.124
Recent Updates in the Management of Advanced Pancreatic Neuroendocrine Tumors
Chang Min Cho1 ,2
Department of Internal Medicine, School of Medicine, Kyungpook National University1; Center for Pancreatobiliary Tumors, Kyungpook National University Chilgok Hospital2, Daegu, Korea
Correspondence to: Chang Min Cho, Center for Pancreatobiliary Tumors, Kyungpook National University Chilgok Hospital, 807 Hokuk-ro, Buk-gu, Daegu 41404, Korea. Tel: +82-53-200-2608, Fax: +82-53-200-2028, E-mail: cmcho@knu.ac.kr, ORCID: https://orcid.org/0000-0002-9903-1282
Received: January 31, 2019; Revised: February 7, 2019; Accepted: February 14, 2019; Published online: March 25, 2019.
© The Korean Journal of Gastroenterology. All rights reserved.

This is an open access article distributed under the terms of the Creative Commons Attribution Non-Commercial License (http://creativecommons.org/licenses/by-nc/4.0) which permits unrestricted non-commercial use, distribution, and reproduction in any medium, provided the original work is properly cited.
Abstract
Pancreatic neuroendocrine tumors (pNETs) are rare neoplasms arising from the pancreatic islet of Langerhans and can be functioning or non-functioning based on the clinical symptoms caused by hormonal secretions. PNETs are the second most common tumor of the pancreas and represent 1-2% of all pancreatic neoplasms. The incidence of pNETs appears to be rising and the prognosis seems to be improving, likely due to the improved treatment options. Recent updates of the World Health Organization classification and grading separate pNETs into 2 broad categories according to the histopathologic criteria, including the Ki-67 proliferative index and mitotic counts: well-differentiated NET and poorly-differentiated neuroendocrine carcinoma (NEC). The classification also incorporates a new subcategory of well-differentiated high-grade NEC (grade 3) to the well-differentiated NET category. This new classification algorithm aims to improve the prediction of the clinical outcomes and survival and help clinicians select better therapeutic strategies for patient care and management. The treatment of advanced or metastatic pNETs may include surgical resection, liver-directed therapies, and/or systemic treatments. In unresectable patients, the goals of these therapies are to palliate the tumor-related symptoms and prolong the lifespan. Systemic therapy consists of the following broad modalities: somatostatin analogues, molecular targeted therapy, systemic chemotherapy, and peptide receptor radionuclide therapy. In conclusion, pNETs are diagnosed increasingly throughout the world, usually with metastatic disease and requiring systemic therapy. Each patient should be evaluated thoroughly and discussed individually by a multidisciplinary and dedicated NET-expert team, which might consider all treatment options, including ongoing clinical trials before selecting the appropriate treatment sequence.
Keywords: Pancreas; Neuroendocrine tumors; Therapy


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