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消化系统混合性神经内分泌-非神经内分泌肿瘤诊断的实用提示

Practical hints for the diagnosis of mixed neuroendocrine-non-neuroendocrine neoplasms of the digestive system.

作者信息

Mattiolo Paola

机构信息

Department of Diagnostics and Public Health, Section of Pathology, University of Verona, University and Hospital Trust of Verona, Verona 37134, Italy.

Department of Pathology, Heinrich Heine University and University Hospital of Duesseldorf, Duesseldorf 40225, Germany.

出版信息

World J Gastrointest Oncol. 2024 Nov 15;16(11):4326-4332. doi: 10.4251/wjgo.v16.i11.4326.

DOI:10.4251/wjgo.v16.i11.4326
PMID:39554731
原文链接:https://pmc.ncbi.nlm.nih.gov/articles/PMC11551634/
Abstract

In this editorial, a comment on the article by Díaz-López published in the recent issue of the 2024 is provided. We focus on the practical implications critical for providing a correct and complete diagnosis of mixed neuroendocrine-non-neuroendocrine neoplasm (MiNEN) in the gastrointestinal system. The diagnosis of MiNEN begins with the recognition of neuroendocrine features in one component of a biphasic tumor. The non-neuroendocrine counterpart can be virtually represented by any neoplastic type, even though the most frequent histologies are glandular and squamous. However, qualification of the neuroendocrine component requires histological and immunohistochemical confirmation. Neuroendocrine tumors are characterized by a peculiar architectural organization and bland nuclei with granular "salt and pepper" chromatin. Although neuroendocrine carcinomas have multiple and variable presentations, they typically show a solid or organoid architecture. The histological aspect needs to be confirmed by immunohistochemistry, and a diagnosis is confirmed whenever the expression of keratin and neuroendocrine markers is observed. Once both histopathological and immunohistochemical features of neuroendocrine neoplasms are identified, it is important to consider the three major pitfalls of MiNEN diagnostics: (1) Entrapment of neuroendocrine non-neoplastic cells within the tumor mass; (2) Differential diagnosis with amphicrine neoplasms; and (3) Differential diagnosis of tumors that partially express neuroendocrine markers. According to the current guidelines for diagnosing digestive MiNEN, each component must represent at least 30% of the entire neoplastic mass. Although the high-grade histopathological subtype frequently determines disease prognosis, both components can significantly affect prognosis. Thus, if one of the components, either neuroendocrine or non-neuroendocrine, does not fulfill the volumetric criteria, the guidelines still encourage reporting it. These strict criteria are essential for correctly recognizing and characterizing digestive MiNENs. This task is essential because it has prognostic relevance and substantial potential value for guiding further studies in this field. In the future, systematic analyses should be performed to validate or reconsider the current 30% cutoff value.

摘要

在这篇社论中,对迪亚兹 - 洛佩斯发表于2024年最新一期的文章进行了评论。我们关注对胃肠道系统中混合性神经内分泌 - 非神经内分泌肿瘤(MiNEN)进行正确、完整诊断至关重要的实际意义。MiNEN的诊断始于识别双相肿瘤一个成分中的神经内分泌特征。非神经内分泌成分实际上可以由任何肿瘤类型代表,尽管最常见的组织学类型是腺性和鳞状。然而,神经内分泌成分的鉴定需要组织学和免疫组织化学确认。神经内分泌肿瘤的特征是具有独特的结构组织和带有颗粒状“椒盐”染色质的淡染细胞核。虽然神经内分泌癌有多种不同表现,但它们通常呈现实体或类器官结构。组织学特征需要通过免疫组织化学确认,当观察到角蛋白和神经内分泌标志物的表达时,诊断得以确认。一旦确定了神经内分泌肿瘤的组织病理学和免疫组织化学特征,重要的是要考虑MiNEN诊断的三个主要陷阱:(1)肿瘤块内神经内分泌非肿瘤细胞的包埋;(2)与双分泌肿瘤的鉴别诊断;(3)部分表达神经内分泌标志物的肿瘤的鉴别诊断。根据目前诊断消化性MiNEN的指南,每个成分必须至少占整个肿瘤块的30%。虽然高级别组织病理学亚型常常决定疾病预后,但两个成分都可显著影响预后。因此,如果神经内分泌或非神经内分泌成分之一不符合体积标准,指南仍鼓励报告。这些严格标准对于正确识别和表征消化性MiNEN至关重要。这项任务至关重要,因为它具有预后相关性以及对该领域进一步研究的重大潜在价值。未来,应进行系统分析以验证或重新考虑当前30%的临界值。

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