摘要
NUT癌(NUT Carcinoma)是一种罕见的起源不明的具有高度侵袭性的恶性肿瘤,其以伴有NUTM1基因染色体重排为主要特点,病理表现为具有突然和局灶性鳞状分化低分化或未分化癌,FISH检测见NUTM1基因重排可明确诊断,NUT癌预后较差,临床上并未明确治疗方案,治疗方式多为手术、放疗及化疗.本文报道1例74岁鼻腔鼻窦NUT癌患者,临床表现为右侧鼻塞伴面部肿胀,鼻窦CT及MRI示右侧鼻腔及上颌窦软组织密度影伴骨质破坏,入院后行鼻窦肿物活检术,术后病理FISH染色结果示BRD4/NUT融合t(15,19),予序贯同步放化疗2个疗程后肿物显著减小,2个月后肿物再次迅速增长侵及硬腭影响进食,行上颌骨部分切除术,术后2个月患者因肿瘤转移累及全身脏器衰竭死亡,生存期11个月,现对本病例的临床特征和诊疗经过进行报告及相关文献复习.
Abstract
NUT Carcinoma(NC)is a rare malignant tumor of unknown origin,which is highly aggressive.It is characterized by chromosome rearrangement accompanied by NUTM1 gene.The pathological manifestations were sudden and focal squamous in poorly differentiated or undifferentiated carcinoma.NUTM1gene rearrange-ment can be used to diagnose NC.The prognosis of NUT cancer is poor.Clinically,there is no established treat-ment plan.treatment options mainly comprise surgery,radiotherapy and chemotherapy.A 74-year-old patient with NC of the nasal cavity and sinuses was reported.Her clinical presentation was right nasal congestion with fa-cial swelling.Sinus CT and MRI showed soft tissue density in the right nasal cavity and maxillary sinus with bone destruction.After admission,the patient underwent nasal endoscopic biopsy,and the postoperative pathological FISH staining showed BRD4/NUT fusion t(15,19).The tumor was significantly reduced after two courses of se-quential chemoradiotherapy.Two months later,the patient underwent a partial maxillary resection due to the rap-id regrowth of sinusoidal mass,invading the hard palate.The patient died 2 months after surgery due to multiple organ failure resulted from tumor metastasis,with a survival time of 11 months.The clinical characteristics,di-agnosis and treatment of this case were reported and related literature was reviewed.
基金项目
中央高水平医院临床科研业务费专项中日友好医院"菁英计划"人才培育工程项目(ZRJY2021-QM03)
中央高水平医院临床科研业务项目(2022-NHLH-CRF-YGJE-02)
NETL
NSTL
万方数据