Several Considerations on the Pathology, Treatment, and Prognosis of Laryngeal NUT Carcinoma.

IF 3.4 Q2 ONCOLOGY

Oncology and Therapy Pub Date : 2026-03-01 DOI:10.1007/s40487-026-00422-1

Andrés Coca-Pelaz, Alfio Ferlito

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Abstract

Laryngeal NUT carcinoma (NC) is an exceptionally rare and highly aggressive malignancy defined by rearrangements of the NUTM1 gene, most commonly involving BRD4. Owing to its morphologic overlap with poorly differentiated squamous cell carcinoma, this entity is frequently underrecognized in routine practice, particularly in the larynx. In this editorial, we summarize current knowledge on the pathology, molecular biology, treatment strategies, and prognosis of laryngeal NC, with particular emphasis on diagnostic pitfalls and clinical outcomes reported in literature. Available evidence highlights a strikingly aggressive clinical course, with most patients experiencing early disease progression and death within the first year of diagnosis, despite multimodal treatment approaches. Rare cases of long-term survival suggest underlying biological heterogeneity, potentially related to fusion subtype. Given the absence of standardized treatment protocols, most reported patients have been managed with an aggressive multimodal approach combining surgery and (chemo)radiation; however, outcomes remain poor. Therefore, early recognition and systematic NUT immunohistochemical testing in poorly differentiated laryngeal carcinomas, especially in younger patients, are critical. Advances in molecularly targeted therapies may offer future opportunities to improve outcomes in this devastating disease.

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喉癌的病理、治疗和预后的几点考虑。

喉部NUT癌（NC）是一种罕见且高度侵袭性的恶性肿瘤，由NUTM1基因重排所定义，最常涉及BRD4。由于其形态与低分化鳞状细胞癌重叠，在常规实践中，这种实体经常被低估，特别是在喉部。在这篇社论中，我们总结了目前关于喉NC的病理学、分子生物学、治疗策略和预后的知识，特别强调了诊断缺陷和文献报道的临床结果。现有证据强调了一个显著的侵袭性临床过程，尽管采用了多种治疗方法，但大多数患者在诊断后的第一年内经历了早期疾病进展和死亡。罕见的长期存活病例表明潜在的生物学异质性，可能与融合亚型有关。由于缺乏标准化的治疗方案，大多数报道的患者都采用积极的多模式方法，结合手术和（化疗）放疗；然而，结果仍然很糟糕。因此，早期识别和系统的NUT免疫组织化学检测对于低分化喉癌，特别是年轻患者，是至关重要的。分子靶向治疗的进展可能为改善这种毁灭性疾病的预后提供未来的机会。

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来源期刊

Oncology and Therapy ONCOLOGY-

CiteScore

3.40

自引率

0.00%

发文量

审稿时长

6 weeks

期刊介绍： Now indexed in PubMed Aims and Scope Oncology and Therapy is an international, peer reviewed, rapid-publication (peer review in 2 weeks, published 3–4 weeks from acceptance) journal dedicated to the publication of high-quality pre-clinical, clinical (all phases), observational, real-world, and health outcomes research around the discovery, development, and use of therapeutics and interventions (including devices) across all therapeutic areas. Studies relating to diagnostics and diagnosis, pharmacoeconomics, public health, epidemiology, quality of life, and patient care, management, and education are also encouraged. The journal is of interest to a broad audience of healthcare professionals and publishes original research, reviews, communications and letters. The journal is read by a global audience and receives submissions from all over the world. Oncology and Therapy will consider all scientifically sound research be it positive, confirmatory or negative data. 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