摘要
目的探讨嗅癌(olfactory carcinoma)的临床病理学特征、诊断及鉴别诊断。方法收集中国科学技术大学附属第一医院(安徽省立医院)临床病理中心2016年1月至2022年8月初诊为嗅神经母细胞瘤(olfactory neuroblastoma,ONB)或不能确定诊断的鼻腔鼻窦肿瘤共21例,其中3例复诊为嗅癌,并以其余18例肿瘤作为观察对照。结果21例病例中3例嗅癌患者,男性2例,女性1例,发病年龄35~74岁,平均年龄57岁。镜下见瘤细胞以实性、巢状或分叶状生长方式为主,周围常见丰富的纤维血管性间质。肿瘤内可见菊形团或假菊形团结构,局部见明显的神经纤维性基质成分。部分癌巢周围细胞呈栅栏状排列。瘤细胞主要呈短梭形或椭圆形,界限不清,胞质少至中等,细胞核染色质较细腻,1个至数个小核仁,核分裂象易见。3例嗅癌均见呈轻度异型腺体,部分腺腔内见纤毛。肿瘤至少弥漫性表达1种上皮性标志物,且同时表达神经内分泌标志物。瘤细胞部分表达p40及p63,癌巢周围的支持细胞不同程度表达S-100蛋白。肿瘤细胞不表达NUT、CD99和结蛋白,SMARCA4(BRG1)和SMARCB1(INI-1)未见表达缺失。Ki-67阳性指数20%~80%。随访16~18个月,均未出现死亡,1例患者16个月后复发。结论嗅癌属于罕见的伴神经上皮分化的鼻腔鼻窦肿瘤,其组织学形态多样,诊断时需密切结合形态及免疫组织化学标记结果。
Objective To investigate the clinicopathological features and differential diagnosis of olfactory carcinoma(OC).Methods Twenty-one cases of sinonasal tumors,including those initially diagnosed as olfactory neuroblastoma(ONB)and those with uncertain diagnosis,were collected from the Department of Pathology,the First Affiliated Hospital of University of Science and Technology of China(Anhui Provincial Hospital)from January 2016 to August 2022,among which 3 cases were reclassified as OC.The clinicopathological features were investigated,and the remaining 18 cases were used as control.Results Of the three OC patients,2 were male and 1 was female,with an average age of 57 years ranging from 35 to 74 years.Microscopically,the tumor cells were arranged in solid,nested or lobulated patterns with occasional palisading around the solid nests.The stroma was highly vascular with focal neurofibrillary areas.There were prominent rosettes or pseudorosettes formation.The tumor cells were mainly ovoid to spindly with scant to moderate amount of cytoplasm,one or several small nucleoli,and fine chromatin content.Brisk mitotic figures were seen.In all 3 cases of OC,there were scanty atypical glands and some were ciliated.Immunohistochemically,at least one epithelial marker and neuroendocrine marker were diffusely expressed in the tumor.Some of the tumor cells were positive for p40 and p63,and the sustentacular cells showed the expression of S-100 protein.All cases tested were negative for NUT,CD99 and desmin,with intact expression of SMARCA4(BRG1)and SMARCB1(INI-1).Ki-67 proliferation index varied from 20%to 80%.Follow-up after 16-18 months showed no mortality with tumor recurrence from 1 patient after 16 months.Conclusion OC is a rare sinonasal tumor with neuroepithelial differentiation,its histomorphology is diverse,and the combination of immunohistochemical markers is essential for appropriate diagnosis.
作者
张晨晨
李恒
程岚卿
吴海波
Zhang Chenchen;Li Heng;Cheng Lanqing;Wu Haibo(Center of Clinical Pathology,the First Affiliated Hospital of University of Science and Technology of China/Anhui Provincial Hospital,University of Science and Technology of China,Hefei 230036,China)
出处
《中华病理学杂志》
CAS
CSCD
北大核心
2023年第11期1138-1143,共6页
Chinese Journal of Pathology
