Recent Updates in the Management of Advanced Pancreatic Neuroendocrine Tumors
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 pancrea...
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| Format: | Article |
| Language: | English |
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Jin Publishing & Printing Co.
2019-03-01
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| Series: | The Korean Journal of Gastroenterology |
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| Online Access: | http://www.kjg.or.kr/journal/view.html?doi=10.4166/kjg.2019.73.3.124 |
| _version_ | 1828451228155117568 |
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| author | Chang Min Cho |
| author_facet | Chang Min Cho |
| author_sort | Chang Min Cho |
| collection | DOAJ |
| description | 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. |
| first_indexed | 2024-12-10T23:34:03Z |
| format | Article |
| id | doaj.art-d6633e67285045d288af8404b12efbe2 |
| institution | Directory Open Access Journal |
| issn | 1598-9992 |
| language | English |
| last_indexed | 2024-12-10T23:34:03Z |
| publishDate | 2019-03-01 |
| publisher | Jin Publishing & Printing Co. |
| record_format | Article |
| series | The Korean Journal of Gastroenterology |
| spelling | doaj.art-d6633e67285045d288af8404b12efbe22022-12-22T01:29:16ZengJin Publishing & Printing Co.The Korean Journal of Gastroenterology1598-99922019-03-0173312413110.4166/kjg.2019.73.3.124kjg.2019.73.3.124Recent Updates in the Management of Advanced Pancreatic Neuroendocrine TumorsChang Min Cho0Department of Internal Medicine, School of Medicine, Kyungpook National University, Daegu, KoreaPancreatic 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.http://www.kjg.or.kr/journal/view.html?doi=10.4166/kjg.2019.73.3.124PancreasNeuroendocrine tumorsTherapy |
| spellingShingle | Chang Min Cho Recent Updates in the Management of Advanced Pancreatic Neuroendocrine Tumors The Korean Journal of Gastroenterology Pancreas Neuroendocrine tumors Therapy |
| title | Recent Updates in the Management of Advanced Pancreatic Neuroendocrine Tumors |
| title_full | Recent Updates in the Management of Advanced Pancreatic Neuroendocrine Tumors |
| title_fullStr | Recent Updates in the Management of Advanced Pancreatic Neuroendocrine Tumors |
| title_full_unstemmed | Recent Updates in the Management of Advanced Pancreatic Neuroendocrine Tumors |
| title_short | Recent Updates in the Management of Advanced Pancreatic Neuroendocrine Tumors |
| title_sort | recent updates in the management of advanced pancreatic neuroendocrine tumors |
| topic | Pancreas Neuroendocrine tumors Therapy |
| url | http://www.kjg.or.kr/journal/view.html?doi=10.4166/kjg.2019.73.3.124 |
| work_keys_str_mv | AT changmincho recentupdatesinthemanagementofadvancedpancreaticneuroendocrinetumors |