Head and neck NUT carcinoma-radiotherapy dose, chemotherapy choice, and outcomes: lessons from a 7-year complete remission.

IF 2.8 3区 医学 Q3 ONCOLOGY
Strahlentherapie und Onkologie Pub Date : 2026-10-01 Epub Date: 2026-06-01 DOI:10.1007/s00066-026-02552-x
Lara Schulz, Matthias Zähringer, Klaus-Henning Kahl, Nikolaos Balagiannis, Bertram Jehs, Johannes Doescher, Johannes Zenk, Georg Stüben, Maria Neu
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引用次数: 0

Abstract

Background: NUT carcinoma (NUTc) is a rare, aggressive squamous carcinoma defined by NUTM1 rearrangements. Outcomes in head and neck (H&N) disease are poor, and no uniform treatment standard exists; available evidence largely derives from case reports and small series.

Methods: We performed a structured narrative review of H&N NUTc with an emphasis on radiotherapy (RT) dose concepts, chemotherapy regimens, and outcome signals.

Results: Published evidence indicates poor overall survival, particularly in metastatic disease. Long-term remissions have been described in selected patients with non-metastatic disease treated with multimodal approaches combining surgery, multi-agent chemotherapy, and early integrated RT. Across reports, use of RT in first-line management and delivery of definitive-dose RT are repeatedly associated with better outcomes. Evidence for immune checkpoint inhibitors is limited to anecdotal single-patient reports.

Case vignette: A 19-year-old man with locally advanced supraglottic NUTc and bilateral cervical nodal disease (M0) presented with dysphagia and otalgia. Following laser debulking and temporary tracheostomy, induction cisplatin/doxorubicin/ifosfamide led to a complete metabolic response in positron-emission tomography/computed tomography (PET/CT) after two cycles of chemotherapy. Definitive PET/CT-guided intensity-modulated (IM)RT/image-guided (IG)RT was delivered to 73.2 Gy using an accelerated twice-daily schedule (2.0 Gy to the planning target volume [PTV] in the morning and 1.6 Gy boost to the initial gross target volume [GTV] in the afternoon; ≥ 8-hour interval). Acute toxicity was grade 3 dermatitis and grade 2 mucositis (CTCAE v5.0). After RT, chemotherapy was continued as consolidation and completed with vincristine/doxorubicin/ifosfamide (six cycles) due to acute kidney injury. Complete remission is ongoing at > 7 years, with persistent xerostomia and dysgeusia.

Conclusion: In selected non-metastatic H&N NUTc, durable control has been reported with timely systemic therapy plus curative-intent RT, often exceeding 50 Gy. This review summarizes dose concepts and case-based systemic regimens and provides a comparative table to support individualized treatment planning.

头颈部NUT癌:放疗剂量、化疗选择和结果:来自7年完全缓解的经验教训。
背景:NUT癌(NUTc)是一种罕见的侵袭性鳞状癌,由NUTM1重排定义。头颈部疾病(H&N)预后较差,且没有统一的治疗标准;现有证据主要来自病例报告和小系列。方法:我们对H&N NUTc进行了结构化的叙述性回顾,重点是放疗(RT)剂量概念,化疗方案和结果信号。结果:已发表的证据表明总生存率较低,特别是在转移性疾病中。在一些非转移性疾病患者中,通过多模式联合手术、多药化疗和早期综合放疗治疗,长期缓解已经得到了描述。在所有报告中,在一线治疗中使用放疗和给予最终剂量放疗反复与更好的结果相关。免疫检查点抑制剂的证据仅限于个别患者的报告。病例简介:一名19岁男性,患有局部晚期声门上NUTc和双侧宫颈结病(M0),表现为吞咽困难和耳痛。在激光减容和临时气管切开术后,诱导顺铂/阿霉素/异环磷酰胺在两个化疗周期后的正电子发射断层扫描/计算机断层扫描(PET/CT)中导致完全的代谢反应。最终PET/ ct引导的调强放射治疗(IM) /图像引导(IG)放射治疗采用每日两次的加速方案(上午2.0 Gy至计划靶体积[PTV],下午1.6 Gy至初始总靶体积[GTV],间隔≥ 8小时),至73.2 Gy。急性毒性为3级皮炎和2级粘膜炎(CTCAE v5.0)。放疗后,由于急性肾损伤,继续化疗作为巩固,并以长春新碱/阿霉素/异环磷酰胺(6个周期)完成。在> 完全缓解持续7年,伴有持续性口干和发音困难。结论:在选定的非转移性H&N NUTc中,据报道,通过及时的全身治疗加上治疗意图的RT,持久的控制通常超过50 Gy。这篇综述总结了剂量概念和基于病例的系统方案,并提供了一个比较表来支持个体化治疗计划。
本文章由计算机程序翻译,如有差异,请以英文原文为准。
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来源期刊
CiteScore
5.70
自引率
12.90%
发文量
141
审稿时长
3-8 weeks
期刊介绍: Strahlentherapie und Onkologie, published monthly, is a scientific journal that covers all aspects of oncology with focus on radiooncology, radiation biology and radiation physics. The articles are not only of interest to radiooncologists but to all physicians interested in oncology, to radiation biologists and radiation physicists. The journal publishes original articles, review articles and case studies that are peer-reviewed. It includes scientific short communications as well as a literature review with annotated articles that inform the reader on new developments in the various disciplines concerned and hence allow for a sound overview on the latest results in radiooncology research. Founded in 1912, Strahlentherapie und Onkologie is the oldest oncological journal in the world. Today, contributions are published in English and German. All articles have English summaries and legends. The journal is the official publication of several scientific radiooncological societies and publishes the relevant communications of these societies.
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