Gao Wen, Feng Lifei, Zhao Xinming, Huang Zishi, Chen Duoxuan, Yin Gaofei, Zhang Yang, Huang Zhigang
Department of Otolaryngology Head and Neck Surgery, Key Laboratory of Otolaryngology Head and Neck Surgery, Ministry of Education, Beijing Tongren Hospital, Capital Medical University, 1, DongJiaoMinXiang Street, Beijing, 100730, China.
Eur Arch Otorhinolaryngol. 2025 Jan;282(1):377-386. doi: 10.1007/s00405-024-08898-1. Epub 2024 Aug 17.
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.
睾丸核蛋白（NUT）癌的特征是15号染色体上的NUT基因重排。本研究的目的是专门探讨鼻窦NUT癌的临床特征、免疫组化、治疗、诊断和预后。
回顾性分析10例经病理确诊的NUT癌患者的临床资料，并复习相关文献。
10例患者中，男性6例，女性4例。中位年龄为34岁（15 - 69岁）。9例患者表现为局部晚期cT4a期。最常见的治疗方法是完整切除联合放疗、化疗和靶向治疗。所有10例患者病理检查均为低分化或未分化癌。此外，免疫组化染色显示10例患者的NUT蛋白均为阳性，大多数病例表达p63、p40和CK。8例患者的Ki-67阳性指数为40%至80%，中位值为50%，其余2例通过荧光原位杂交（FISH）检测到NUTM1基因断裂。截至2023年4月，所有患者均随访1 - 51个月，中位随访时间为14个月。3例患者因广泛的全身转移死亡，3例复发，4例无复发或转移。
鼻窦NUT癌是一种罕见且侵袭性很强的恶性肿瘤，进展迅速，预后较差。正确的组织病理学诊断是确定适当治疗的首要前提。目前NUT癌尚无有效的治疗方案。靶向治疗可能成为治疗NUT癌的有效方法。
