Fonseca Daphne, Kodandapani Suseela, Babu Rayala Vikas, Rao Chandrasekhara, Rao Subramanyeshwar
Department of Laboratory Medicine, Basavatarakam Indo American Cancer Hospital and Research Institute, Hyderabad, Telangana State 500034 India.
Department of Pathology, Basavatarakam Indo American Cancer Hospital and Research Institute, Hyderabad, Telangana State 500034 India.
Indian J Otolaryngol Head Neck Surg. 2023 Sep;75(3):2355-2358. doi: 10.1007/s12070-023-03691-8. Epub 2023 Mar 20.
Nuclear protein of the testis (NUT) carcinoma is a rare and aggressive malignancy associated with rearrangements of the NUT gene on chromosome 15q14 .This entity is often under diagnosed and there is limited literature regarding its biology and optimal management. We report a case of pediatric NUT carcinoma presenting as a mass involving the nasal septum with associated bilateral cervical lymphadenopathy. CT and MRI of PNS revealed soft tissue lesion involving cartilaginous and bony septum and tip of nose .Histopathology of cervical lymph node and IHC with NUT confirmed the diagnosis of metastatic NUT carcinoma. NUT carcinoma has a dismal prognosis despite aggressive multimodality management. As new NUT targeting drugs using BET inhibitors are emerging, the correct and prompt recognition of NC is the key to improve patient outcome.
睾丸核蛋白（NUT）癌是一种罕见的侵袭性恶性肿瘤，与15号染色体q14区域的NUT基因重排有关。该实体常被漏诊，关于其生物学特性和最佳治疗方法的文献有限。我们报告一例儿童NUT癌，表现为累及鼻中隔并伴有双侧颈部淋巴结肿大的肿块。鼻旁窦的CT和MRI显示软组织病变累及软骨性和骨性鼻中隔及鼻尖。颈部淋巴结的组织病理学检查和NUT免疫组化证实为转移性NUT癌。尽管采用了积极的多模式治疗，NUT癌的预后仍很差。随着使用溴结构域和超末端结构域（BET）抑制剂的新型NUT靶向药物的出现，正确及时地识别NUT癌是改善患者预后的关键。
