33例嗅神经母细胞瘤临床病理及预后分析  

Clinicopathological and prognostic analysis of 33 patients with olfactory neuroblastoma

作　　者：王玉姣 王佳鹤 尚轶钒 陈伶俐 黄思夏 李烨[1] 王蕾[1] 吕新全[1] WANG Yujiao;WANG Jiahe;SHANG Yifan;CHEN Lingli;HUANG Sixia;LI Ye;WANG Lei;LYU Xinquan(Department of Pathology,the First Affiliated Hospital of Zhengzhou University,Zhengzhou 450052,China)

机构地区：[1]郑州大学第一附属医院病理科,河南郑州450052

出　　处：《肿瘤基础与临床》2023年第3期213-219,共7页journal of basic and clinical oncology

基　　金：河南省高等学校重点科研计划项目(22A310023)。

摘　　要：目的探讨嗅神经母细胞瘤的临床病理特征及预后情况。方法回顾性纳入2013年10月至2021年7月郑州大学第一附属医院病理科确诊的33例嗅神经母细胞瘤患者,其中男27例(81.82%)、女6例(18.18%),分析患者临床资料、组织病理学特征和免疫表型,比较不同Kadish分期和Hyams分级患者预后情况。结果33例嗅神经母细胞瘤患者中,51~60岁占比最高,主要临床症状为鼻出血、鼻塞,Kadish分期主要为C期(36.36%)和D期(45.45%),其中淋巴结转移患者14例(2例伴骨转移)、单纯远处骨转移者1例,Hyams分级主要为Ⅱ级(33.33%)和Ⅲ级(39.39%)。光镜下可见,肿瘤位于黏膜下,被丰富纤维血管间质分隔呈分叶状结构(75.76%),肿瘤细胞间有不等量的神经原纤维(66.67%),假菊形团结构一般见于低级别肿瘤中(30.30%),腺样分化结构在高级别与低级别肿瘤中均可见,肿瘤细胞为形态一致的小蓝圆细胞,“胡椒盐样”的染色质为其特有改变。免疫组化染色突触素阳性96.97%(32/33),CD56阳性87.88%(29/33),细胞角蛋白阳性或部分阳性69.70%(23/33),嗜铬蛋白A多为微弱点状阳性54.55%(18/33),S-100阳性48.48%(16/33),Ki-67≥20%占78.79%(26/33),神经胶质纤维酸性蛋白多为阴性表达。截止到2022年11月,2例失访,Kadish B期、C期、D期患者5 a生存率分别为100.00%(5/5)、63.64%(7/11)、60.00%(9/15),差异无统计学意义(χ^(2)=3.339,P=0.118);Hyams分级中,低级别和高级别患者生存率分别为78.57%(11/14),58.82%(10/17),差异无统计学意义(χ^(2)=1.303,P=0.254)。结论嗅神经母细胞瘤的组织病理学有特征性改变,结合免疫表型有助于临床诊断,Kadish分期和Hyams分级在嗅神经母细胞瘤患者预后评估中有一定的参考价值。Objective To investigate the clinicopathologic features and prognosis of olfactory neuroblastoma.Methods A total of 33 olfactory neuroblastoma patients diagnosed in the First Affiliated Hospital of Zhengzhou University from October 2013 to July 2021 were retrospectively included,including 27 males(81.82%)and 6 females(18.18%).The clinical data,histopathological characteristics and immunophenotype of the patients were analyzed.The prognosis of patients with different Kadish stages and Hyams grades were analyzed.Results Of the 33 patients with olfactory neuroblastoma,51-60 years of age accounted for the highest proportion;the main clinical symptoms were nasal bleeding and nasal congestion;Kadish stages were mainly stage C(36.36%)and stage D(45.45%);14 patients had lymph node metastasis(2 patients with bone metastasis),1 patient had simple distant bone metastasis;the main grades of Hyams were gradeⅡ(33.33%)and gradeⅢ(39.39%).Under light microscope,the tumor was located in the submucosa and was separated by rich fibrous vascular interstroma in lobular structure(75.76%),and there were unequal amounts of neurofibrils between tumor cells(66.67%).Pseudochrysanthemum-shaped mass structure was generally seen in low-grade tumors(30.30%),and adenoid differentiated structure was seen in both high and low grade tumors.The tumor cells were small round blue cells with uniform morphology,and the chromatin of"pepper salt"was its characteristic change.Immunohistochemical staining was positive for synaptophysin 96.97%(32/33),CD5687.88%positive(29/33),cytokeratin positive 69.70%(23/33),chromogranin A mostly weak spot positive 54.55%(18/33),S-100 positive 48.48%(16/33),78.79%(26/33)had Ki-67≥20%,and glial fibrillary acidic protein was mostly negative.By November 2022,2 patients were lost to follow-up.The 5-year survival rates of Kadish stage B,C and D patients were 100.00%(5/5),63.64%(7/11),60.00%(9/15),respectively.There was no significant difference in the survival rate among patients with different Kadish stages(χ^(2)=3.339,

关 键 词：嗅神经母细胞瘤 临床病理特征 预后 

分 类 号：R739.4[医药卫生—肿瘤]