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Pancreatic Adeno-MiNEN, a Rare Newly Defined Entity with Challenging Diagnosis and Treatment: A Case Report with Systematic Literature Review and Pooled Analysis
Mixed neuroendocrine non-neuroendocrine neoplasms (MiNEN) are a peculiar entity that can occur throughout the whole gastrointestinal trait, and pancreatic localization is rare. Their main characteristic is the presence of at least a neuroendocrine and an epithelial component, each accounting for at...
Autores principales: | , , , , , , , , , , , |
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Formato: | Online Artículo Texto |
Lenguaje: | English |
Publicado: |
MDPI
2022
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Materias: | |
Acceso en línea: | https://www.ncbi.nlm.nih.gov/pmc/articles/PMC9457227/ https://www.ncbi.nlm.nih.gov/pubmed/36078951 http://dx.doi.org/10.3390/jcm11175021 |
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author | Angelico, Roberta Siragusa, Leandro Pathirannehalage Don, Cristine Brooke Sensi, Bruno Billeci, Federica Vattermoli, Leonardo Padial, Belen Palmieri, Giampiero Anselmo, Alessandro Coppola, Alessandro Tisone, Giuseppe Manzia, Tommaso Maria |
author_facet | Angelico, Roberta Siragusa, Leandro Pathirannehalage Don, Cristine Brooke Sensi, Bruno Billeci, Federica Vattermoli, Leonardo Padial, Belen Palmieri, Giampiero Anselmo, Alessandro Coppola, Alessandro Tisone, Giuseppe Manzia, Tommaso Maria |
author_sort | Angelico, Roberta |
collection | PubMed |
description | Mixed neuroendocrine non-neuroendocrine neoplasms (MiNEN) are a peculiar entity that can occur throughout the whole gastrointestinal trait, and pancreatic localization is rare. Their main characteristic is the presence of at least a neuroendocrine and an epithelial component, each accounting for at least 30% of the tumour mass. The presence of epithelial ductal component defines adeno-MiNEN. We report a case of a 59-year-old woman affected by pancreatic adeno-MiNEN with challenging diagnosis and successfully treated. A systematic literature review and pooled analysis was also performed, aiming to define the management and outcomes of pancreatic adeno-MiNEN. Out of 190 identified records, 15 studies including 28 patients affected by pancreatic-adeno-MiNEN were included in the analysis. Pancreatic adeno-MiNEN occurred mainly in males (82.8%) and at a mean age of 61.7 (range: 24–82) years. Pre-operative diagnosis was possible only in 14.2% of cases. At presentation, the majority had already advanced disease (TNM stage III (53.8%) and stage IV 19.3%). Adjuvant therapy was performed in 55% of patients, and the tumour recurrence rate was in 30% of cases. Median disease-free survival (DFS) was 12 months (range: 0–216 months) with a 5-year DFS of 16.6%, while the median overall survival (OS) was 12 months (range: 0–288 months) with a 5-year OS of 23.5%. Pancreatic adeno-MiNENs are rare; as they have very heterogenous behaviour, they are rarely diagnosed preoperatively and have poor prognosis. Treatment of localised MiNEN still relies on radical surgical resection, which seems essential to achieve a good oncological prognosis. International registry on MiNEN is necessary to improve the knowledge on this rare tumour and to improve its outcomes. |
format | Online Article Text |
id | pubmed-9457227 |
institution | National Center for Biotechnology Information |
language | English |
publishDate | 2022 |
publisher | MDPI |
record_format | MEDLINE/PubMed |
spelling | pubmed-94572272022-09-09 Pancreatic Adeno-MiNEN, a Rare Newly Defined Entity with Challenging Diagnosis and Treatment: A Case Report with Systematic Literature Review and Pooled Analysis Angelico, Roberta Siragusa, Leandro Pathirannehalage Don, Cristine Brooke Sensi, Bruno Billeci, Federica Vattermoli, Leonardo Padial, Belen Palmieri, Giampiero Anselmo, Alessandro Coppola, Alessandro Tisone, Giuseppe Manzia, Tommaso Maria J Clin Med Review Mixed neuroendocrine non-neuroendocrine neoplasms (MiNEN) are a peculiar entity that can occur throughout the whole gastrointestinal trait, and pancreatic localization is rare. Their main characteristic is the presence of at least a neuroendocrine and an epithelial component, each accounting for at least 30% of the tumour mass. The presence of epithelial ductal component defines adeno-MiNEN. We report a case of a 59-year-old woman affected by pancreatic adeno-MiNEN with challenging diagnosis and successfully treated. A systematic literature review and pooled analysis was also performed, aiming to define the management and outcomes of pancreatic adeno-MiNEN. Out of 190 identified records, 15 studies including 28 patients affected by pancreatic-adeno-MiNEN were included in the analysis. Pancreatic adeno-MiNEN occurred mainly in males (82.8%) and at a mean age of 61.7 (range: 24–82) years. Pre-operative diagnosis was possible only in 14.2% of cases. At presentation, the majority had already advanced disease (TNM stage III (53.8%) and stage IV 19.3%). Adjuvant therapy was performed in 55% of patients, and the tumour recurrence rate was in 30% of cases. Median disease-free survival (DFS) was 12 months (range: 0–216 months) with a 5-year DFS of 16.6%, while the median overall survival (OS) was 12 months (range: 0–288 months) with a 5-year OS of 23.5%. Pancreatic adeno-MiNENs are rare; as they have very heterogenous behaviour, they are rarely diagnosed preoperatively and have poor prognosis. Treatment of localised MiNEN still relies on radical surgical resection, which seems essential to achieve a good oncological prognosis. International registry on MiNEN is necessary to improve the knowledge on this rare tumour and to improve its outcomes. MDPI 2022-08-26 /pmc/articles/PMC9457227/ /pubmed/36078951 http://dx.doi.org/10.3390/jcm11175021 Text en © 2022 by the authors. https://creativecommons.org/licenses/by/4.0/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 (https://creativecommons.org/licenses/by/4.0/). |
spellingShingle | Review Angelico, Roberta Siragusa, Leandro Pathirannehalage Don, Cristine Brooke Sensi, Bruno Billeci, Federica Vattermoli, Leonardo Padial, Belen Palmieri, Giampiero Anselmo, Alessandro Coppola, Alessandro Tisone, Giuseppe Manzia, Tommaso Maria Pancreatic Adeno-MiNEN, a Rare Newly Defined Entity with Challenging Diagnosis and Treatment: A Case Report with Systematic Literature Review and Pooled Analysis |
title | Pancreatic Adeno-MiNEN, a Rare Newly Defined Entity with Challenging Diagnosis and Treatment: A Case Report with Systematic Literature Review and Pooled Analysis |
title_full | Pancreatic Adeno-MiNEN, a Rare Newly Defined Entity with Challenging Diagnosis and Treatment: A Case Report with Systematic Literature Review and Pooled Analysis |
title_fullStr | Pancreatic Adeno-MiNEN, a Rare Newly Defined Entity with Challenging Diagnosis and Treatment: A Case Report with Systematic Literature Review and Pooled Analysis |
title_full_unstemmed | Pancreatic Adeno-MiNEN, a Rare Newly Defined Entity with Challenging Diagnosis and Treatment: A Case Report with Systematic Literature Review and Pooled Analysis |
title_short | Pancreatic Adeno-MiNEN, a Rare Newly Defined Entity with Challenging Diagnosis and Treatment: A Case Report with Systematic Literature Review and Pooled Analysis |
title_sort | pancreatic adeno-minen, a rare newly defined entity with challenging diagnosis and treatment: a case report with systematic literature review and pooled analysis |
topic | Review |
url | https://www.ncbi.nlm.nih.gov/pmc/articles/PMC9457227/ https://www.ncbi.nlm.nih.gov/pubmed/36078951 http://dx.doi.org/10.3390/jcm11175021 |
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