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Evidence of a common cell origin in a case of pancreatic mixed intraductal papillary mucinous neoplasm–neuroendocrine tumor
Virchows Archiv ( IF 3.4 ) Pub Date : 2020-10-01 , DOI: 10.1007/s00428-020-02942-1
Marco Schiavo Lena 1 , Maria Giulia Cangi 1 , Lorenza Pecciarini 1 , Ilaria Francaviglia 1 , Greta Grassini 1 , Renaud Maire 2 , Stefano Partelli 3 , Massimo Falconi 3 , Aurel Perren 2 , Claudio Doglioni 1
Affiliation  

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.



中文翻译:

胰腺混合管内乳头状黏液性肿瘤-神经内分泌肿瘤的常见细胞起源证据

近来,已经提出了术语混合神经内分泌-非神经内分泌肿瘤(MiNEN)作为涵盖了混合神经内分泌-外分泌肿瘤的不同可能组合的总括定义。其中,腺瘤与神经内分泌肿瘤(NET)的组合是最稀有的一种,尚未被2019年WHO分类标准正式认可。在这种情况下,碰撞肿瘤或真正的混合肿瘤之间的争论仍未解决。在此报告中,描述了胰管内乳头状黏液性肿瘤(IPMN)和NET,并且分子研究显示两个种群中都存在相同的KRAS,GNAS和CDKN2A突变,以及CCND1基因的扩增。这些数据证明了克隆性,并支持这两种成分的共同起源,因此证实了真正的混合性质。为此原因,

更新日期:2020-10-02
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