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起源于声门下喉的大细胞神经内分泌癌。

Large cell neuroendocrine carcinoma originating in the subglottic larynx.

作者信息

Elton Andrew, Van Beck Jenna, Li Bin, Ondrey Frank G

机构信息

University of Minnesota Medical School, Minneapolis, MN, USA.

Department of Otolaryngology, Head and Neck Surgery, University of Minnesota, Minneapolis, MN, USA.

出版信息

Ear Nose Throat J. 2025 Feb;104(2):NP46-NP49. doi: 10.1177/01455613211054628. Epub 2022 Apr 27.

Abstract

Large cell neuroendocrine carcinoma (LCNEC) of the larynx is an exceedingly rare cancer of the head and neck that is difficult to diagnose. Few case reports of laryngeal LCNEC exist within the literature, and it was not until recently that LCNEC was recognized as a discrete subtype of neuroendocrine carcinoma. Given its recent recognition as a distinct subtype, histologic characteristics distinguishing LCNEC from other poorly differentiated carcinomas remain under investigation. Various reports have shown genetic alterations such as p53 and/or p16 overexpression, which are typically associated with infection by human papilloma virus (HPV). However, some reports have shown p53 and/or p16 overexpression in HPV negative samples. In this case, we discuss a 67-year-old patient with a history of extensive alcohol and tobacco use with a newly diagnosed T4N0M0, high grade, LCNEC of the subglottic larynx. Tumor pathology demonstrated positive staining for typical neuroendocrine (NE) markers like synaptophysin and chromogranin A; however, there was diffuse CK34βE12 and p16 expression. LCNEC is a newly classified subtype of poorly differentiated neuroendocrine (NE) tumors, and the diagnosis requires consideration of the clinical presentation, microscopic features, and immunostaining markers.

摘要

喉大细胞神经内分泌癌(LCNEC)是一种极其罕见的头颈部癌症,难以诊断。文献中关于喉LCNEC的病例报告很少,直到最近LCNEC才被确认为神经内分泌癌的一个独立亚型。鉴于其最近被确认为一个独特的亚型,区分LCNEC与其他低分化癌的组织学特征仍在研究中。各种报告显示了基因改变,如p53和/或p16过表达,这些通常与人乳头瘤病毒(HPV)感染有关。然而,一些报告显示在HPV阴性样本中也存在p53和/或p16过表达。在此病例中,我们讨论一名67岁有大量饮酒和吸烟史的患者,其新诊断为声门下喉T4N0M0、高级别LCNEC。肿瘤病理显示典型神经内分泌(NE)标志物如突触素和嗜铬粒蛋白A染色阳性;然而,存在弥漫性CK34βE12和p16表达。LCNEC是一种新分类的低分化神经内分泌(NE)肿瘤亚型,诊断需要考虑临床表现、微观特征和免疫染色标志物。

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