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

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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.

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Acknowledgments

The authors want to thank Dr. Luca Albarello for useful discussion and suggestions, Roberto Cairella and Patricia Ney for their invaluable technical assistance.

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MF and SP were involved in patient care and provided clinical information and specimen. LP and IF performed FISH. RM performed laser capture microdissection. MGC and GG performed sequencing. MSL, AP, and CD wrote the manuscript.

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Correspondence to Marco Schiavo Lena.

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The authors declare that they have no conflict of interest.

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Schiavo Lena, M., Cangi, M.G., Pecciarini, L. et al. Evidence of a common cell origin in a case of pancreatic mixed intraductal papillary mucinous neoplasm–neuroendocrine tumor. Virchows Arch 478, 1215–1219 (2021). https://doi.org/10.1007/s00428-020-02942-1

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  • DOI: https://doi.org/10.1007/s00428-020-02942-1

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