摘要
目的探讨SMARCB1缺失型鼻腔鼻窦腺癌临床病理学特征。方法收集6例SMARCB1缺失型鼻腔鼻窦腺癌临床病理资料,观察及分析其临床病理特征,并文献复习。结果6例均为男性,年龄34~78岁,中位年龄60岁,镜下肿瘤细胞形态较均匀一致,呈嗜酸细胞样5例(5/6)、浆细胞样3例(3/6)、印戒细胞样及透明细胞样各1例(1/6),可见筛状结构4例(4/6)、小管状及卵黄囊瘤样结构各1例(1/6),黏液样基质常见(5/6)。免疫组化染色示6例均对SMARCB1表达缺失,5例对SMARCA2表达缺失、1例表达减弱,ARID1A及SMARCB4完整,4例中度表达CK7(4/6)、灶状表达p63及SALL4各2例(2/6),不表达CDX2、PLAP及S100;3例进行FISH检测,均可见SMARCB1基因缺失;随访4例患者,3例于术后16个月内死亡,1例复发3次生存。结论SMARCB1缺失型鼻腔鼻窦腺癌是一种少见的恶性肿瘤,预后差,结合组织学形态及免疫组化染色有助于鉴别诊断。
Objective To investigate the clinicopathological features of primary SMARCB1-deficient sinonasal adenocarcinomas(SNACs).Methods The clinicopathological data of 6 cases of SMARCB1-deficient SNACs were collected,and the clinicopathological characteristics were observed and analyzed,and related literatures were reviewed.Results Six patients were male,aged 34-78 years,with a median age of 60 years.The tumor cells were uniform,showing oncocytoid in 5 cases(5/6),plasmacytoid in 3 cases(3/6),signet-ring and clear cell like in 1 case,respectively(1/6).Tubules in the form of punched-out luminal spaces and yolk sac tumor like were noted in 1 case(1/6),cribriform in 4 cases(4/6)and yolk sac tumor like structures(1/6),and mucoid matrix was commonly found(5/6).Immunohistochemical staining showed that all cases were completely negative for SMARCB1,5 cases shown SMARCA2 loss,and 1 case was weak expression,while ARID1A and SMARCB4 were retained,with moderate expression for CK7(4/6)and focal expression for p63(2/6)and Sall4(2/6),and no expression for CDX2,PLAP and S100.The SMARB1 gene deletion was detected in 3 cases by FISH.Among the 4 cases followed up,3 cases died within 16 months after operation,and 1 case recurred in three times and survived.Conclusion SMARCB1-deficient SNAC is rare and its prognosis is poor.Combination of histological morphology and immunohistochemistry is helpful for its differential diagnosis.
作者
岳常丽
熊焰
刘红刚
朴颖实
YUE Chang-li;XIONG Yan;LIU Hong-gang;PIAO Ying-shi(Department of Pathology,Beijing Tongren Hospital,Capital Medical University,Beijing 100730,China;Department of Pathology,Peking University First Hospital,Beijing 100034,China)
出处
《诊断病理学杂志》
2025年第3期263-267,共5页
Chinese Journal of Diagnostic Pathology
关键词
鼻腔鼻窦
SMARCB1缺失型腺癌
临床病理学特征
鉴别诊断
Nasal cavity and paranasal sinus
SMARCB1-deficient adenocarcinoma
Clinicopathological features
Differential diagnosis
