Wang Lei, Zhu Zhenzhen, Wang Weiqing, Zha Yang, Wang Xiaowei, Surita Aodeng, Liu Yuzhuo, Lv Wei
Department of Otolaryngology-Head and Neck Surgery, Peking Union Medical College Hospital, Chinese Academy of Medical Sciences, Peking Union Medical College, Beijing, China.
Front Surg. 2023 Mar 1;10:1098704. doi: 10.3389/fsurg.2023.1098704. eCollection 2023.
Nuclear protein in testis (NUT) carcinoma is a rare, aggressive tumor defined by the presence of gene rearrangement. The aim of this study was to describe the clinical, radiologic, and biological features of sinonasal NUT carcinoma.
We retrospectively investigated NUT expression with clinicopathologic features in 145 cases with sinonasal malignancies diagnosed from January 2017 to December 2021 and reviewed the reported cases.
Three (3/145, 2.07%) cases showed strong nuclear expression for NUT immunohistochemical, including one male and two females with ages from 37 to 57 years (mean, 45.33 years). All three cases involved the nasal cavity and sinuses; one of them involved the orbit and intracranial area. Histologically, all subjects showed poorly differentiated, small round cell morphology with distinct nuclei. All patients received surgery and chemoradiotherapy. One patient died of the disease 13 months after diagnosis, and two survived 12 and 15 months, respectively, without evidence of tumor recurrence. 51 cases of sinonasal NUT carcinoma (mean age 40.96 years) have been described to date. Among them, 28 are male, and 23 are female. Most cases expressed p63, AE1/AE3, as well as p40.
NUT carcinoma is a rare and aggressive disease with a poor prognosis. It is crucial to perform rearrangement-related tests for differential diagnosis of poorly differentiated/undifferentiated tumors in the nasal cavity and sinuses.
睾丸核蛋白（NUT）癌是一种罕见的侵袭性肿瘤，由基因重排的存在所定义。本研究的目的是描述鼻窦NUT癌的临床、放射学和生物学特征。
我们回顾性研究了2017年1月至2021年12月诊断的145例鼻窦恶性肿瘤中NUT表达与临床病理特征，并复习了已报道的病例。
3例（3/145，2.07%）病例NUT免疫组化显示强核表达，包括1例男性和2例女性，年龄37至57岁（平均45.33岁）。所有3例均累及鼻腔和鼻窦；其中1例累及眼眶和颅内区域。组织学上，所有受试者均表现为低分化的小圆形细胞形态，核明显。所有患者均接受了手术及放化疗。1例患者在诊断后13个月死于该疾病，2例分别存活12个月和15个月，无肿瘤复发迹象。迄今为止，已描述了51例鼻窦NUT癌（平均年龄40.96岁）。其中，男性28例，女性23例。大多数病例表达p63、AE1/AE3以及p40。
NUT癌是一种罕见且侵袭性强、预后差的疾病。对鼻腔和鼻窦低分化/未分化肿瘤进行重排相关检测以进行鉴别诊断至关重要。
