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Case Reports
. 2021 Jun;478(6):1215-1219.
doi: 10.1007/s00428-020-02942-1. Epub 2020 Oct 1.

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

Marco Schiavo Lena  1 , Maria Giulia Cangi  2 , Lorenza Pecciarini  2 , Ilaria Francaviglia  2 , Greta Grassini  2 , Renaud Maire  3 , Stefano Partelli  4 , Massimo Falconi  4 , Aurel Perren  3 , Claudio Doglioni  2
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Free article
Case Reports

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

Marco Schiavo Lena et al. Virchows Arch. 2021 Jun.
Free article

Abstract

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.

Keywords: CDKN2A mutation; Cyclin D1 amplification; Intraductal papillary mucinous neoplasm; KRAS and GNAS mutation; Mixed neuroendocrine non-neuroendocrine neoplasms; Pancreatic neuroendocrine tumor.

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