Chemotherapy in Well Differentiated Neuroendocrine Tumors (NET) G1, G2, and G3: A Narrative Review

Neuroendocrine tumors (NETs) are rare neoplasms with a wide spectrum of clinical behavior, from the long survival of well-differentiated NETs to the dismal prognosis of high-grade neuroendocrine carcinomas (NECs), being G3 NETs a recently recognized intermediate entity. While the role of chemotherap...

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Main Authors: Arianna Zappi, Irene Persano, Linda Galvani, Elena Parlagreco, Elisa Andrini, Davide Campana, Maria Pia Brizzi, Giuseppe Lamberti, Anna La Salvia
Format: Article
Language:English
Published: MDPI AG 2023-01-01
Series:Journal of Clinical Medicine
Subjects:
Online Access:https://www.mdpi.com/2077-0383/12/2/717
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author Arianna Zappi
Irene Persano
Linda Galvani
Elena Parlagreco
Elisa Andrini
Davide Campana
Maria Pia Brizzi
Giuseppe Lamberti
Anna La Salvia
author_facet Arianna Zappi
Irene Persano
Linda Galvani
Elena Parlagreco
Elisa Andrini
Davide Campana
Maria Pia Brizzi
Giuseppe Lamberti
Anna La Salvia
author_sort Arianna Zappi
collection DOAJ
description Neuroendocrine tumors (NETs) are rare neoplasms with a wide spectrum of clinical behavior, from the long survival of well-differentiated NETs to the dismal prognosis of high-grade neuroendocrine carcinomas (NECs), being G3 NETs a recently recognized intermediate entity. While the role of chemotherapy is well established in NECs, data on NETs mostly derives from small studies, experts’ opinions, and extrapolating results from small-cell lung cancer studies. This narrative review aims to summarize available evidence about the use of chemotherapy in the setting of G1-2 NETs and G3 NETs. We performed literature research in PubMed Library for all articles published up to September 2022 about the efficacy of chemotherapy in NETs. Treatment regimens with STZ-5FU, CAPTEM, and anti-metabolite-based treatment are the most active and tolerated in gastroenteropancreatic NETs (GEP-NETs) G1-G2, while platinum-based regimens (FOLFOX/XELOX) and TEM/CAPTEM showed the best activity in thoracic NETs. Solid evidence about chemotherapy efficacy in G3 NETs is still lacking. Literature data support the use of chemotherapy in low-intermediate grade NETs after the failure of other therapies or if tumor shrinkage is needed. Studies assessing G3 NETs independently from NECs are needed to better understand the role of chemotherapy in this setting.
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spelling doaj.art-14afb42d8cb64d8a92c8166a2ef786f62023-11-30T22:53:53ZengMDPI AGJournal of Clinical Medicine2077-03832023-01-0112271710.3390/jcm12020717Chemotherapy in Well Differentiated Neuroendocrine Tumors (NET) G1, G2, and G3: A Narrative ReviewArianna Zappi0Irene Persano1Linda Galvani2Elena Parlagreco3Elisa Andrini4Davide Campana5Maria Pia Brizzi6Giuseppe Lamberti7Anna La Salvia8Department of Specialized, Experimental and Diagnostic Medicine, S. Orsola-Malpighi Hospital, University of Bologna, 40126 Bologna, ItalyDepartment of Oncology, San Luigi Gonzaga Hospital, 10043 Orbassano, ItalyDepartment of Specialized, Experimental and Diagnostic Medicine, S. Orsola-Malpighi Hospital, University of Bologna, 40126 Bologna, ItalyDepartment of Oncology, San Luigi Gonzaga Hospital, 10043 Orbassano, ItalyDepartment of Specialized, Experimental and Diagnostic Medicine, S. Orsola-Malpighi Hospital, University of Bologna, 40126 Bologna, ItalyDepartment of Specialized, Experimental and Diagnostic Medicine, S. Orsola-Malpighi Hospital, University of Bologna, 40126 Bologna, ItalyDepartment of Oncology, San Luigi Gonzaga Hospital, 10043 Orbassano, ItalyDepartment of Specialized, Experimental and Diagnostic Medicine, S. Orsola-Malpighi Hospital, University of Bologna, 40126 Bologna, ItalyNational Center for Drug Research and Evaluation, National Institute of Health (ISS), 00161 Rome, ItalyNeuroendocrine tumors (NETs) are rare neoplasms with a wide spectrum of clinical behavior, from the long survival of well-differentiated NETs to the dismal prognosis of high-grade neuroendocrine carcinomas (NECs), being G3 NETs a recently recognized intermediate entity. While the role of chemotherapy is well established in NECs, data on NETs mostly derives from small studies, experts’ opinions, and extrapolating results from small-cell lung cancer studies. This narrative review aims to summarize available evidence about the use of chemotherapy in the setting of G1-2 NETs and G3 NETs. We performed literature research in PubMed Library for all articles published up to September 2022 about the efficacy of chemotherapy in NETs. Treatment regimens with STZ-5FU, CAPTEM, and anti-metabolite-based treatment are the most active and tolerated in gastroenteropancreatic NETs (GEP-NETs) G1-G2, while platinum-based regimens (FOLFOX/XELOX) and TEM/CAPTEM showed the best activity in thoracic NETs. Solid evidence about chemotherapy efficacy in G3 NETs is still lacking. Literature data support the use of chemotherapy in low-intermediate grade NETs after the failure of other therapies or if tumor shrinkage is needed. Studies assessing G3 NETs independently from NECs are needed to better understand the role of chemotherapy in this setting.https://www.mdpi.com/2077-0383/12/2/717chemotherapywell differentiated neuroendocrine neoplasmsneuroendocrine tumorstreatment
spellingShingle Arianna Zappi
Irene Persano
Linda Galvani
Elena Parlagreco
Elisa Andrini
Davide Campana
Maria Pia Brizzi
Giuseppe Lamberti
Anna La Salvia
Chemotherapy in Well Differentiated Neuroendocrine Tumors (NET) G1, G2, and G3: A Narrative Review
Journal of Clinical Medicine
chemotherapy
well differentiated neuroendocrine neoplasms
neuroendocrine tumors
treatment
title Chemotherapy in Well Differentiated Neuroendocrine Tumors (NET) G1, G2, and G3: A Narrative Review
title_full Chemotherapy in Well Differentiated Neuroendocrine Tumors (NET) G1, G2, and G3: A Narrative Review
title_fullStr Chemotherapy in Well Differentiated Neuroendocrine Tumors (NET) G1, G2, and G3: A Narrative Review
title_full_unstemmed Chemotherapy in Well Differentiated Neuroendocrine Tumors (NET) G1, G2, and G3: A Narrative Review
title_short Chemotherapy in Well Differentiated Neuroendocrine Tumors (NET) G1, G2, and G3: A Narrative Review
title_sort chemotherapy in well differentiated neuroendocrine tumors net g1 g2 and g3 a narrative review
topic chemotherapy
well differentiated neuroendocrine neoplasms
neuroendocrine tumors
treatment
url https://www.mdpi.com/2077-0383/12/2/717
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