Evidence of a common cell origin in a case of pancreatic mixed intraductal papillary mucinous neoplasm–neuroendocrine tumor

Recently, the term mixed neuroendocrine non-neuroendocrine neoplasms (MiNEN) has been proposed as an umbrella definition covering different possible combinations of mixed neuroendocrine-exocrine neoplasms. Among these, the adenoma plus neuroendocrine tumor (NET) combination is among the rarest and n...

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Veröffentlicht in:Virchows Archiv : an international journal of pathology Jg. 478; H. 6; S. 1215 - 1219
Hauptverfasser: Schiavo Lena, Marco, Cangi, Maria Giulia, Pecciarini, Lorenza, Francaviglia, Ilaria, Grassini, Greta, Maire, Renaud, Partelli, Stefano, Falconi, Massimo, Perren, Aurel, Doglioni, Claudio
Format: Journal Article
Sprache:Englisch
Veröffentlicht: Berlin/Heidelberg Springer Berlin Heidelberg 01.06.2021
Springer Nature B.V
Schlagworte:
Adenoma
Brief Report
CCND1 gene
Medicine
Medicine & Public Health
Mutation
Neoplasms
Neuroendocrine tumors
Pancreas
Pancreatic cancer
Pathology
Tumors
Intraductal papillary mucinous neoplasm
Mixed neuroendocrine non-neuroendocrine neoplasms
KRAS and GNAS mutation
CDKN2A mutation
Cyclin D1 amplification
Pancreatic neuroendocrine tumor
ISSN:0945-6317, 1432-2307, 1432-2307
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Zusammenfassung:Recently, the term mixed neuroendocrine non-neuroendocrine neoplasms (MiNEN) has been proposed as an umbrella definition covering different possible combinations of mixed neuroendocrine-exocrine neoplasms. Among these, the adenoma plus neuroendocrine tumor (NET) combination is among the rarest and not formally recognized by the 2019 WHO Classification. In this setting, the debate between either collision tumors or true mixed neoplasms is still unsolved. In this report, a pancreatic intraductal papillary mucinous neoplasm (IPMN) plus a NET is described, and the molecular investigations showed the presence in both populations of the same KRAS, GNAS, and CDKN2A mutations and the amplification of the CCND1 gene. These data prove clonality and support a common origin of both components, therefore confirming the true mixed nature. For this reason, mixed neuroendocrine-exocrine neoplasms, in which the exocrine component is represented by a glandular precursor lesion (adenoma/IPMN) only, should be included into the MiNEN family.
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ISSN:0945-6317
1432-2307
1432-2307
DOI:10.1007/s00428-020-02942-1