睾丸核蛋白（NUT）癌以15号染色体上NUT基因重排为特征。本研究旨在探讨鼻腔鼻窦NUT癌的临床特征、免疫组化、治疗、诊断和预后。10例NUT的临床资料对经病理证实的癌症进行回顾性分析，并查阅相关文献。10例患者中，男性6例，女性4例。中位年龄为 34 岁（15-69 岁）。 9 名患者出现局部晚期 cT4a 期。最常见的治疗方法是完全切除联合放疗、化疗和靶向治疗。所有10名患者病理学上均患有低分化或未分化癌。免疫组化结果显示，10例患者NUT蛋白均呈阳性，大部分病例表达p63、p40和CK。 8例患者的Ki-67阳性指数范围为40%至80%，中位值为50%，其余2例均通过FISH检测到NUTM1基因破坏。截至 2023 年 4 月，所有患者均接受了 1-51 个月的随访，中位随访时间为 14 个月。 3例患者因全身广泛转移而死亡，3例复发，4例未复发或转移。鼻窦NCs（NUT癌）是一种罕见的、高度侵袭性的恶性肿瘤，进展快，预后差。正确的组织病理学诊断是确定适当治疗的首要前提。目前对于 NC 尚无有效的治疗方案。靶向治疗可能成为治疗 NC 的有效方法。© 2024。作者获得 Springer-Verlag GmbH 德国（Springer Nature 旗下公司）的独家许可。

Nuclear protein in testis (NUT) carcinoma is characterized by NUT gene rearrangement on chromosome 15. The objective of this study was to investigate the clinical features, immunohistochemistry, treatment, diagnosis and prognosis of sinonasal NUT carcinoma specifically.Clinical data for 10 cases of NUT cancer confirmed by pathology were retrospectively analyzed, and the relevant literature was reviewed.Among the 10 patients, 6 were males, and 4 were females. The median age was 34 years (15-69 years). Nine patients presented with locally advanced cT4a stage. The most common treatment was complete resection combined with radiotherapy, chemotherapy, and targeted therapy. All 10 patients had pathologically poorly differentiated or undifferentiated carcinoma. Furthermore, immunohistochemical staining showed that NUT protein was positive in all 10 patients, and most cases expressed p63, p40 and CK. The Ki-67 positive index of 8 patients ranged from 40 to 80%, with a median of 50%, and NUTM1 gene disruption was detected in both of the remaining cases by FISH. As of April, 2023, all patients were followed up with for 1-51 months, with a median follow-up time of 14 months. Three patients died due to widespread systemic metastasis, 3 relapsed, and 4 had no recurrence or metastasis.Sinonasal NCs (NUT carcinomas) is a rare and highly aggressive malignant tumor with rapid progression and a poor prognosis. Correct histopathological diagnosis is the primary prerequisite for determining appropriate treatment. There are currently no effective treatment options for NCs. Targeted therapy may become an effective method to treat NCs.© 2024. The Author(s), under exclusive licence to Springer-Verlag GmbH Germany, part of Springer Nature.