Michelle Yoon, Ricardo Ramirez, Harrison Smith, Luke Stanisce, Soo Yeon Sohn, Margaret Brandwein-Weber, Mark Urken
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引用次数: 0
Abstract
Background
NUT midline carcinoma (NMC) is an aggressive malignancy driven by NUTM1 fusions in poorly differentiated tumor cells. This pooled analysis evaluates the impact of primary tumor location on overall survival (OS) in head and neck (HN) NMC.
Methods
A systematic review of case reports was performed. Kaplan–Meier survival analysis was used to assess OS differences by subsite, age, and genetic profile.
Results
Of 261 screened articles, 81 met inclusion criteria. Overall, 138 cases were included in demographic analyzes and 65 in survival analyzes. Tracheo-laryngopharyngeal tumors trended toward worse OS (HR 2.45, 95% CI 1.01–5.95, p = 0.047). Orbito-sinonasal tumors trended toward improved OS (HR 0.75, 95% CI 0.46–1.24, p = 0.25). BRD4-NUTM1 fusions were associated with worse OS (HR 8.3, 95% CI 4.26–16.23, p = 0.0003).
Conclusions
HN NMC carries a poor prognosis, and BRD4-NUTM1 fusions have worse outcomes. Primary subsite is a potential prognostic variable that may inform future studies.
背景:NUT中线癌(NMC)是一种在低分化肿瘤细胞中由NUTM1融合驱动的侵袭性恶性肿瘤。本汇总分析评估了原发肿瘤位置对头颈部NMC总生存期(OS)的影响。方法:对病例报告进行系统回顾。Kaplan-Meier生存分析用于评估亚位点、年龄和遗传谱的OS差异。结果:261篇筛选的文章中,81篇符合纳入标准。总体而言,人口统计学分析纳入138例,生存分析纳入65例。气管-喉咽肿瘤有恶化OS的趋势(HR 2.45, 95% CI 1.01-5.95, p = 0.047)。眼眶-鼻窦肿瘤有改善OS的趋势(HR 0.75, 95% CI 0.46-1.24, p = 0.25)。BRD4-NUTM1融合与较差的OS相关(HR 8.3, 95% CI 4.26-16.23, p = 0.0003)。结论:HN NMC预后较差,BRD4-NUTM1融合预后较差。原发性子位点是一个潜在的预后变量,可以为未来的研究提供信息。
期刊介绍:
Head & Neck is an international multidisciplinary publication of original contributions concerning the diagnosis and management of diseases of the head and neck. This area involves the overlapping interests and expertise of several surgical and medical specialties, including general surgery, neurosurgery, otolaryngology, plastic surgery, oral surgery, dermatology, ophthalmology, pathology, radiotherapy, medical oncology, and the corresponding basic sciences.