嗅神经母细胞瘤10例临床病理分析

A Clinicopathologic Analysis of 10 Cases of Olfactory Neuroblastoma

目的:探讨嗅神经母细胞瘤(olfactory neuroblastoma,ONB)的临床病理学特点、鉴别诊断,提高嗅神经母细胞瘤的病理诊断水平。方法:回顾性分析本院2016年2月-2020年5月10例ONB的苏木精-伊红染色法(hematoxylin-eosin staining,HE)形态及免疫组织化学染色,结合文献分析其临床表现、病理学形态特点及免疫表型。结果:10例均为Kadish分期C期,HE染色示肿瘤细胞大小形态一致,小圆形或卵圆形,细胞质稀少,核膜不清,被明显的纤维血管性间质分割,呈小叶状结构,可见Homer-Wrignt型假菊形团或Flexner Wintersteriner型真菊形团,免疫组织化学示肿瘤细胞一致性表达Syn、NSE,S-100表达于肿瘤细胞巢周支持细胞。结论:ONB是一种罕见的鼻腔、鼻窦及颅底的恶性肿瘤,起病隐匿,发现时多已是晚期,生物学行为与组织学特征多样,与鼻腔、鼻窦其他恶性肿瘤有重叠,尚无特异的分子标记物,寻找特征性组织学表现,辅以免疫组化及分子检测排除其他小圆细胞恶性肿瘤有助于确诊。

Objective:To investigate the clinical pathology characteristics and differential diagnosis of olfactory neuroblastoma(ONB)in order to improve the pathological diagnosis of olfactory neuroblastoma.Method:From February 2016 to May 2020,the hematoxylin-eosin staining(HE)morphology and immunohistochemical staining in 10 cases of ONB in our hospital were analyzed retrospectively.The clinical manifestations,pathological features and immunophenotypes were analyzed combined with the literature.Result:Ten patients were Kadish stage:stage C.HE staining showed that the tumor cells were uniform in size and shape,small round or oval in shape,with sparse cytoplasm,unclear nuclear membrane,and separated by obvious fibrovascular Stroma,there was a lobular structure,such as Homer-Wrignt pseudo-chrysanthemum or Flexner Wintersteriner true chrysanthemum.Immunohistochemistry showed consistent expression of Syn,NSE in tumor cells,S-100 was expressed in peri-nest supporting cells of tumor cells.Conclusion:ONB is a rare malignant tumor of nasal cavity,paranasal sinuses and skull base.The onset of ONB is concealed,and it is usually found in advanced stage.Its biological behavior and histological features are various,and it overlaps with other malignant tumors of nasal cavity and paranasal sinuses,no specific molecular marker was found.Finding the characteristic histologic features,combined with immunohistochemistry and molecular detection to exclude other small round cell malignancies would be helpful for the diagnosis.