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...
| Main Authors: | , , , , , , , , , , , |
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| Format: | Article |
| Language: | English |
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MDPI AG
2022-08-01
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| Series: | Journal of Clinical Medicine |
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| Online Access: | https://www.mdpi.com/2077-0383/11/17/5021 |
| _version_ | 1797494830795849728 |
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| author | Roberta Angelico Leandro Siragusa Cristine Brooke Pathirannehalage Don Bruno Sensi Federica Billeci Leonardo Vattermoli Belen Padial Giampiero Palmieri Alessandro Anselmo Alessandro Coppola Giuseppe Tisone Tommaso Maria Manzia |
| author_facet | Roberta Angelico Leandro Siragusa Cristine Brooke Pathirannehalage Don Bruno Sensi Federica Billeci Leonardo Vattermoli Belen Padial Giampiero Palmieri Alessandro Anselmo Alessandro Coppola Giuseppe Tisone Tommaso Maria Manzia |
| author_sort | Roberta Angelico |
| collection | DOAJ |
| 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. |
| first_indexed | 2024-03-10T01:39:57Z |
| format | Article |
| id | doaj.art-faf439dae5184d9ba42e815f2e405242 |
| institution | Directory Open Access Journal |
| issn | 2077-0383 |
| language | English |
| last_indexed | 2024-03-10T01:39:57Z |
| publishDate | 2022-08-01 |
| publisher | MDPI AG |
| record_format | Article |
| series | Journal of Clinical Medicine |
| spelling | doaj.art-faf439dae5184d9ba42e815f2e4052422023-11-23T13:26:18ZengMDPI AGJournal of Clinical Medicine2077-03832022-08-011117502110.3390/jcm11175021Pancreatic Adeno-MiNEN, a Rare Newly Defined Entity with Challenging Diagnosis and Treatment: A Case Report with Systematic Literature Review and Pooled AnalysisRoberta Angelico0Leandro Siragusa1Cristine Brooke Pathirannehalage Don2Bruno Sensi3Federica Billeci4Leonardo Vattermoli5Belen Padial6Giampiero Palmieri7Alessandro Anselmo8Alessandro Coppola9Giuseppe Tisone10Tommaso Maria Manzia11HPB and Transplant Unit, Department of Surgical Sciences, University of Rome Tor Vergata, 00133 Rome, ItalyHPB and Transplant Unit, Department of Surgical Sciences, University of Rome Tor Vergata, 00133 Rome, ItalyHPB and Transplant Unit, Department of Surgical Sciences, University of Rome Tor Vergata, 00133 Rome, ItalyHPB and Transplant Unit, Department of Surgical Sciences, University of Rome Tor Vergata, 00133 Rome, ItalyHPB and Transplant Unit, Department of Surgical Sciences, University of Rome Tor Vergata, 00133 Rome, ItalyDepartment of Diagnostic Imaging and Interventional Radiology, Tor Vergata University of Rome, 00133 Rome, ItalyHistopathologic Unit, Tor Vergata University of Rome, 00133 Rome, ItalyHistopathologic Unit, Tor Vergata University of Rome, 00133 Rome, ItalyHPB and Transplant Unit, Department of Surgical Sciences, University of Rome Tor Vergata, 00133 Rome, ItalyDepartment of Surgery, University Campus Bio-Medico of Rome, 00128 Rome, ItalyHPB and Transplant Unit, Department of Surgical Sciences, University of Rome Tor Vergata, 00133 Rome, ItalyHPB and Transplant Unit, Department of Surgical Sciences, University of Rome Tor Vergata, 00133 Rome, ItalyMixed 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.https://www.mdpi.com/2077-0383/11/17/5021pancreatic tumourmixed neuroendocrine-non neuroendocrine neoplasmMiNEN |
| spellingShingle | Roberta Angelico Leandro Siragusa Cristine Brooke Pathirannehalage Don Bruno Sensi Federica Billeci Leonardo Vattermoli Belen Padial Giampiero Palmieri Alessandro Anselmo Alessandro Coppola Giuseppe Tisone Tommaso Maria Manzia Pancreatic Adeno-MiNEN, a Rare Newly Defined Entity with Challenging Diagnosis and Treatment: A Case Report with Systematic Literature Review and Pooled Analysis Journal of Clinical Medicine pancreatic tumour mixed neuroendocrine-non neuroendocrine neoplasm MiNEN |
| 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 | pancreatic tumour mixed neuroendocrine-non neuroendocrine neoplasm MiNEN |
| url | https://www.mdpi.com/2077-0383/11/17/5021 |
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