探索诊断灰色地带:一例具有挑战性的胰腺高级别神经内分泌肿瘤病例

IF 4.6 Q2 MATERIALS SCIENCE, BIOMATERIALS
Brooke Mullen, Albert L. Sy, Priscila Dias Goncalves, M. Lisa Zhang
{"title":"探索诊断灰色地带:一例具有挑战性的胰腺高级别神经内分泌肿瘤病例","authors":"Brooke Mullen, Albert L. Sy, Priscila Dias Goncalves, M. Lisa Zhang","doi":"10.1186/s13000-024-01546-6","DOIUrl":null,"url":null,"abstract":"Grade 3 neuroendocrine tumor (G3 PanNET) and poorly differentiated neuroendocrine carcinoma (PanNEC) of the pancreas are considered distinct entities from a biological and prognostic perspective but may have overlapping features complicating a definitive diagnosis. A 52-year-old female presented with a pancreatic body mass and liver lesions. Initial biopsies showed variable lower- and higher-grade morphologies and modestly elevated Ki67 proliferation index up to 30%, leading to a diagnosis of G3 PanNET. The patient underwent everolimus treatment followed by surgical resection, revealing a complex tumor with features of both G3 PanNET and PanNEC, including admixed well- and poorly differentiated morphologies, modestly elevated hotspot Ki67 of 28%, retained ATRX/DAXX expression, and loss of RB expression. The final diagnosis rendered was “high-grade neuroendocrine neoplasm” with discussion of both entities in the differential. Post-operatively, the patient remains alive with stable metastases. This case highlights the diagnostic complexities of distinguishing G3 PanNET and PanNEC even with the support of ancillary immunohistochemical and molecular studies. In addition, such cases raise the possibility that G3 PanNET and PanNEC may lie on a spectrum of disease with potential biological overlap.","PeriodicalId":2,"journal":{"name":"ACS Applied Bio Materials","volume":null,"pages":null},"PeriodicalIF":4.6000,"publicationDate":"2024-09-12","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":"0","resultStr":"{\"title\":\"Navigating the diagnostic gray zone: a challenging case of pancreatic high-grade neuroendocrine neoplasm\",\"authors\":\"Brooke Mullen, Albert L. Sy, Priscila Dias Goncalves, M. Lisa Zhang\",\"doi\":\"10.1186/s13000-024-01546-6\",\"DOIUrl\":null,\"url\":null,\"abstract\":\"Grade 3 neuroendocrine tumor (G3 PanNET) and poorly differentiated neuroendocrine carcinoma (PanNEC) of the pancreas are considered distinct entities from a biological and prognostic perspective but may have overlapping features complicating a definitive diagnosis. A 52-year-old female presented with a pancreatic body mass and liver lesions. Initial biopsies showed variable lower- and higher-grade morphologies and modestly elevated Ki67 proliferation index up to 30%, leading to a diagnosis of G3 PanNET. The patient underwent everolimus treatment followed by surgical resection, revealing a complex tumor with features of both G3 PanNET and PanNEC, including admixed well- and poorly differentiated morphologies, modestly elevated hotspot Ki67 of 28%, retained ATRX/DAXX expression, and loss of RB expression. The final diagnosis rendered was “high-grade neuroendocrine neoplasm” with discussion of both entities in the differential. Post-operatively, the patient remains alive with stable metastases. This case highlights the diagnostic complexities of distinguishing G3 PanNET and PanNEC even with the support of ancillary immunohistochemical and molecular studies. In addition, such cases raise the possibility that G3 PanNET and PanNEC may lie on a spectrum of disease with potential biological overlap.\",\"PeriodicalId\":2,\"journal\":{\"name\":\"ACS Applied Bio Materials\",\"volume\":null,\"pages\":null},\"PeriodicalIF\":4.6000,\"publicationDate\":\"2024-09-12\",\"publicationTypes\":\"Journal Article\",\"fieldsOfStudy\":null,\"isOpenAccess\":false,\"openAccessPdf\":\"\",\"citationCount\":\"0\",\"resultStr\":null,\"platform\":\"Semanticscholar\",\"paperid\":null,\"PeriodicalName\":\"ACS Applied Bio Materials\",\"FirstCategoryId\":\"3\",\"ListUrlMain\":\"https://doi.org/10.1186/s13000-024-01546-6\",\"RegionNum\":0,\"RegionCategory\":null,\"ArticlePicture\":[],\"TitleCN\":null,\"AbstractTextCN\":null,\"PMCID\":null,\"EPubDate\":\"\",\"PubModel\":\"\",\"JCR\":\"Q2\",\"JCRName\":\"MATERIALS SCIENCE, BIOMATERIALS\",\"Score\":null,\"Total\":0}","platform":"Semanticscholar","paperid":null,"PeriodicalName":"ACS Applied Bio Materials","FirstCategoryId":"3","ListUrlMain":"https://doi.org/10.1186/s13000-024-01546-6","RegionNum":0,"RegionCategory":null,"ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":null,"EPubDate":"","PubModel":"","JCR":"Q2","JCRName":"MATERIALS SCIENCE, BIOMATERIALS","Score":null,"Total":0}
引用次数: 0

摘要

从生物学和预后角度来看,胰腺 3 级神经内分泌肿瘤(G3 PanNET)和分化不良神经内分泌癌(PanNEC)被认为是不同的实体,但它们可能有重叠的特征,从而使明确诊断变得复杂。一名 52 岁的女性因胰腺体块和肝脏病变就诊。最初的活检显示低级别和高级别形态不一,Ki67增殖指数略有升高,最高达30%,诊断为G3 PanNET。患者接受了依维莫司治疗,随后进行了手术切除,结果发现该肿瘤非常复杂,同时具有 G3 PanNET 和 PanNEC 的特征,包括好分化和差分化形态混杂、热点 Ki67 略微升高 28%、ATRX/DAXX 表达保留以及 RB 表达缺失。最终诊断为 "高级别神经内分泌肿瘤",并在鉴别中讨论了这两种实体。术后,患者仍然存活,转移情况稳定。该病例凸显了区分 G3 PanNET 和 PanNEC 的诊断复杂性,即使有辅助免疫组化和分子研究的支持。此外,此类病例还提出了一种可能性,即G3 PanNET和PanNEC可能处于一个疾病谱上,具有潜在的生物学重叠。
本文章由计算机程序翻译,如有差异,请以英文原文为准。
Navigating the diagnostic gray zone: a challenging case of pancreatic high-grade neuroendocrine neoplasm
Grade 3 neuroendocrine tumor (G3 PanNET) and poorly differentiated neuroendocrine carcinoma (PanNEC) of the pancreas are considered distinct entities from a biological and prognostic perspective but may have overlapping features complicating a definitive diagnosis. A 52-year-old female presented with a pancreatic body mass and liver lesions. Initial biopsies showed variable lower- and higher-grade morphologies and modestly elevated Ki67 proliferation index up to 30%, leading to a diagnosis of G3 PanNET. The patient underwent everolimus treatment followed by surgical resection, revealing a complex tumor with features of both G3 PanNET and PanNEC, including admixed well- and poorly differentiated morphologies, modestly elevated hotspot Ki67 of 28%, retained ATRX/DAXX expression, and loss of RB expression. The final diagnosis rendered was “high-grade neuroendocrine neoplasm” with discussion of both entities in the differential. Post-operatively, the patient remains alive with stable metastases. This case highlights the diagnostic complexities of distinguishing G3 PanNET and PanNEC even with the support of ancillary immunohistochemical and molecular studies. In addition, such cases raise the possibility that G3 PanNET and PanNEC may lie on a spectrum of disease with potential biological overlap.
求助全文
通过发布文献求助,成功后即可免费获取论文全文。 去求助
来源期刊
ACS Applied Bio Materials
ACS Applied Bio Materials Chemistry-Chemistry (all)
CiteScore
9.40
自引率
2.10%
发文量
464
×
引用
GB/T 7714-2015
复制
MLA
复制
APA
复制
导出至
BibTeX EndNote RefMan NoteFirst NoteExpress
×
提示
您的信息不完整,为了账户安全,请先补充。
现在去补充
×
提示
您因"违规操作"
具体请查看互助需知
我知道了
×
提示
确定
请完成安全验证×
copy
已复制链接
快去分享给好友吧!
我知道了
右上角分享
点击右上角分享
0
联系我们:info@booksci.cn Book学术提供免费学术资源搜索服务,方便国内外学者检索中英文文献。致力于提供最便捷和优质的服务体验。 Copyright © 2023 布克学术 All rights reserved.
京ICP备2023020795号-1
ghs 京公网安备 11010802042870号
Book学术文献互助
Book学术文献互助群
群 号:481959085
Book学术官方微信