Somatostatin Analogue Therapy in MEN1-Related Pancreatic Neuroendocrine Tumors from Evidence to Clinical Practice: A Systematic Review

Neuroendocrine neoplasms (NENs) are relatively rare and complex tumors that can be sporadic or hereditary, as in the context of multiple endocrine neoplasia type 1 (MEN1) where patients display a 70% lifelong risk of developing a pancreatic NENs (pNENs). To date, specific personalized treatment for...

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Main Authors: Anna La Salvia, Franz Sesti, Chiara Grinzato, Rossella Mazzilli, Maria Grazia Tarsitano, Elisa Giannetta, Antongiulio Faggiano
Format: Article
Language:English
Published: MDPI AG 2021-10-01
Series:Pharmaceuticals
Subjects:
Online Access:https://www.mdpi.com/1424-8247/14/10/1039
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author Anna La Salvia
Franz Sesti
Chiara Grinzato
Rossella Mazzilli
Maria Grazia Tarsitano
Elisa Giannetta
Antongiulio Faggiano
author_facet Anna La Salvia
Franz Sesti
Chiara Grinzato
Rossella Mazzilli
Maria Grazia Tarsitano
Elisa Giannetta
Antongiulio Faggiano
author_sort Anna La Salvia
collection DOAJ
description Neuroendocrine neoplasms (NENs) are relatively rare and complex tumors that can be sporadic or hereditary, as in the context of multiple endocrine neoplasia type 1 (MEN1) where patients display a 70% lifelong risk of developing a pancreatic NENs (pNENs). To date, specific personalized treatment for pNENs in patients with MEN1 are lacking. The aim of this study was to systematically analyze the efficacy and safety of somatostatin analogue (SSA) treatment in patients affected by MEN1-related pNENs. We performed a systematic review of the literature, searching for peer-reviewed articles on SSA (octreotide or lanreotide) treatment in MEN1 associated with pNENs. We selected 20 studies with a pooled population of 105 MEN1 patients with pNENs. Females were 58.5%, median age was 44 years (18–73). TNM stage at diagnosis was stage I–II in 84.8% and stage IV in 15.2%. The overall response rate (SD+PR+CR) was achieved in 88.3% of cases, with stable disease in 75.6% and objective response in 12.7% of patients. The safety profile was favorable with both SSA agents. SSAs appear to be an effective and safe treatment option for MEN1-related pNEN, either at localized or advanced stages.
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spelling doaj.art-9826a90703e54f0981cd5dc9dec4d6302023-11-22T19:36:32ZengMDPI AGPharmaceuticals1424-82472021-10-011410103910.3390/ph14101039Somatostatin Analogue Therapy in MEN1-Related Pancreatic Neuroendocrine Tumors from Evidence to Clinical Practice: A Systematic ReviewAnna La Salvia0Franz Sesti1Chiara Grinzato2Rossella Mazzilli3Maria Grazia Tarsitano4Elisa Giannetta5Antongiulio Faggiano6Department of Oncology, 12 de Octubre University Hospital, 28045 Madrid, SpainDepartment of Experimental Medicine, Sapienza University of Rome, 00161 Rome, ItalyDepartment of Experimental Medicine, Sapienza University of Rome, 00161 Rome, ItalyDepartment of Clinical and Molecular Medicine, Sant’Andrea Hospital, Sapienza University of Rome, 00189 Rome, ItalyDepartment of Experimental Medicine, Sapienza University of Rome, 00161 Rome, ItalyDepartment of Experimental Medicine, Sapienza University of Rome, 00161 Rome, ItalyDepartment of Experimental Medicine, Sapienza University of Rome, 00161 Rome, ItalyNeuroendocrine neoplasms (NENs) are relatively rare and complex tumors that can be sporadic or hereditary, as in the context of multiple endocrine neoplasia type 1 (MEN1) where patients display a 70% lifelong risk of developing a pancreatic NENs (pNENs). To date, specific personalized treatment for pNENs in patients with MEN1 are lacking. The aim of this study was to systematically analyze the efficacy and safety of somatostatin analogue (SSA) treatment in patients affected by MEN1-related pNENs. We performed a systematic review of the literature, searching for peer-reviewed articles on SSA (octreotide or lanreotide) treatment in MEN1 associated with pNENs. We selected 20 studies with a pooled population of 105 MEN1 patients with pNENs. Females were 58.5%, median age was 44 years (18–73). TNM stage at diagnosis was stage I–II in 84.8% and stage IV in 15.2%. The overall response rate (SD+PR+CR) was achieved in 88.3% of cases, with stable disease in 75.6% and objective response in 12.7% of patients. The safety profile was favorable with both SSA agents. SSAs appear to be an effective and safe treatment option for MEN1-related pNEN, either at localized or advanced stages.https://www.mdpi.com/1424-8247/14/10/1039somatostatin analoguesMEN-1pancreatic neuroendocrine tumorsefficacysafetypersonalized treatment
spellingShingle Anna La Salvia
Franz Sesti
Chiara Grinzato
Rossella Mazzilli
Maria Grazia Tarsitano
Elisa Giannetta
Antongiulio Faggiano
Somatostatin Analogue Therapy in MEN1-Related Pancreatic Neuroendocrine Tumors from Evidence to Clinical Practice: A Systematic Review
Pharmaceuticals
somatostatin analogues
MEN-1
pancreatic neuroendocrine tumors
efficacy
safety
personalized treatment
title Somatostatin Analogue Therapy in MEN1-Related Pancreatic Neuroendocrine Tumors from Evidence to Clinical Practice: A Systematic Review
title_full Somatostatin Analogue Therapy in MEN1-Related Pancreatic Neuroendocrine Tumors from Evidence to Clinical Practice: A Systematic Review
title_fullStr Somatostatin Analogue Therapy in MEN1-Related Pancreatic Neuroendocrine Tumors from Evidence to Clinical Practice: A Systematic Review
title_full_unstemmed Somatostatin Analogue Therapy in MEN1-Related Pancreatic Neuroendocrine Tumors from Evidence to Clinical Practice: A Systematic Review
title_short Somatostatin Analogue Therapy in MEN1-Related Pancreatic Neuroendocrine Tumors from Evidence to Clinical Practice: A Systematic Review
title_sort somatostatin analogue therapy in men1 related pancreatic neuroendocrine tumors from evidence to clinical practice a systematic review
topic somatostatin analogues
MEN-1
pancreatic neuroendocrine tumors
efficacy
safety
personalized treatment
url https://www.mdpi.com/1424-8247/14/10/1039
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