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Therapy of Patients with Neuroendocrine Neoplasia—Evidence-Based Approaches and New Horizons
Neuroendocrine tumors (NETs) show low but increasing incidence and originate in multiple organs, including the pancreas, midgut, caecum, rectum, appendix, colon, and lungs. Due to their stunning genetic, histological, and clinical variability, diagnosis and treatment of NETs are challenging. In addi...
Autores principales: | , , , |
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Formato: | Online Artículo Texto |
Lenguaje: | English |
Publicado: |
MDPI
2019
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Materias: | |
Acceso en línea: | https://www.ncbi.nlm.nih.gov/pmc/articles/PMC6780372/ https://www.ncbi.nlm.nih.gov/pubmed/31527438 http://dx.doi.org/10.3390/jcm8091474 |
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author | Bundschuh, Ralph A. Habacha, Bilel Lütje, Susanne Essler, Markus |
author_facet | Bundschuh, Ralph A. Habacha, Bilel Lütje, Susanne Essler, Markus |
author_sort | Bundschuh, Ralph A. |
collection | PubMed |
description | Neuroendocrine tumors (NETs) show low but increasing incidence and originate in multiple organs, including the pancreas, midgut, caecum, rectum, appendix, colon, and lungs. Due to their stunning genetic, histological, and clinical variability, diagnosis and treatment of NETs are challenging. In addition, low incidence and high variability hamper the implementation of high evidence trials. Therefore, guidelines do not cover the complexity of NETs and, frequently, treatment decisions are taken by interdisciplinary tumor conferences at comprehensive cancer centers. Treatment aims are (i) control of tumor growth, (ii) symptom control, as well as (iii) the improvement of progression-free survival (PFS) and overall survival (OS). Here, we discuss high evidence trials facilitating the achievement of these treatment aims. The majority of the evidence exists for treatment with somatostatin analogue, everolimus, peptide receptor radionuclide therapy (PRRT) with (177)Lu-DOTATATE, sunitinib, and telotristat. Among those, PRRT is the only treatment option that has the potential to control symptoms, stop tumor growth, and to improve PFS and OS. In contrast, only a low level of evidence exists for treatment with cytotoxic drugs such as streptozotocin and doxorubicine. Finally, we discuss novel treatment options by a combination of cytotoxic drugs, (177)Lu-DOTATATE, and tyrosine kinase inhibitors to be tested in randomized prospective trials in the future. In addition, the application of innovative isotopes, such as (225)Ac, for PRRT is discussed. |
format | Online Article Text |
id | pubmed-6780372 |
institution | National Center for Biotechnology Information |
language | English |
publishDate | 2019 |
publisher | MDPI |
record_format | MEDLINE/PubMed |
spelling | pubmed-67803722019-10-30 Therapy of Patients with Neuroendocrine Neoplasia—Evidence-Based Approaches and New Horizons Bundschuh, Ralph A. Habacha, Bilel Lütje, Susanne Essler, Markus J Clin Med Review Neuroendocrine tumors (NETs) show low but increasing incidence and originate in multiple organs, including the pancreas, midgut, caecum, rectum, appendix, colon, and lungs. Due to their stunning genetic, histological, and clinical variability, diagnosis and treatment of NETs are challenging. In addition, low incidence and high variability hamper the implementation of high evidence trials. Therefore, guidelines do not cover the complexity of NETs and, frequently, treatment decisions are taken by interdisciplinary tumor conferences at comprehensive cancer centers. Treatment aims are (i) control of tumor growth, (ii) symptom control, as well as (iii) the improvement of progression-free survival (PFS) and overall survival (OS). Here, we discuss high evidence trials facilitating the achievement of these treatment aims. The majority of the evidence exists for treatment with somatostatin analogue, everolimus, peptide receptor radionuclide therapy (PRRT) with (177)Lu-DOTATATE, sunitinib, and telotristat. Among those, PRRT is the only treatment option that has the potential to control symptoms, stop tumor growth, and to improve PFS and OS. In contrast, only a low level of evidence exists for treatment with cytotoxic drugs such as streptozotocin and doxorubicine. Finally, we discuss novel treatment options by a combination of cytotoxic drugs, (177)Lu-DOTATATE, and tyrosine kinase inhibitors to be tested in randomized prospective trials in the future. In addition, the application of innovative isotopes, such as (225)Ac, for PRRT is discussed. MDPI 2019-09-16 /pmc/articles/PMC6780372/ /pubmed/31527438 http://dx.doi.org/10.3390/jcm8091474 Text en © 2019 by the authors. Licensee MDPI, Basel, Switzerland. This article is an open access article distributed under the terms and conditions of the Creative Commons Attribution (CC BY) license (http://creativecommons.org/licenses/by/4.0/). |
spellingShingle | Review Bundschuh, Ralph A. Habacha, Bilel Lütje, Susanne Essler, Markus Therapy of Patients with Neuroendocrine Neoplasia—Evidence-Based Approaches and New Horizons |
title | Therapy of Patients with Neuroendocrine Neoplasia—Evidence-Based Approaches and New Horizons |
title_full | Therapy of Patients with Neuroendocrine Neoplasia—Evidence-Based Approaches and New Horizons |
title_fullStr | Therapy of Patients with Neuroendocrine Neoplasia—Evidence-Based Approaches and New Horizons |
title_full_unstemmed | Therapy of Patients with Neuroendocrine Neoplasia—Evidence-Based Approaches and New Horizons |
title_short | Therapy of Patients with Neuroendocrine Neoplasia—Evidence-Based Approaches and New Horizons |
title_sort | therapy of patients with neuroendocrine neoplasia—evidence-based approaches and new horizons |
topic | Review |
url | https://www.ncbi.nlm.nih.gov/pmc/articles/PMC6780372/ https://www.ncbi.nlm.nih.gov/pubmed/31527438 http://dx.doi.org/10.3390/jcm8091474 |
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