摘要
目的探讨脂肪神经细胞瘤的临床病理学特征、诊断及鉴别诊断要点。方法选取2011年6月至2020年3月首都医科大学宣武医院病理科和福建医科大学附属第一医院病理科诊断的脂肪神经细胞瘤9例,回顾性分析患者的临床资料、影像学特征、病理学特点、免疫表型及预后。结果9例患者中,男4例,女5例,年龄5~63岁,首发症状为头痛、头晕伴恶心呕吐8例,行走不稳1例。影像学显示,7例病灶位于小脑半球,1例病灶位于侧脑室,均为单发肿块;另有1例病灶位于侧脑室和延髓,为多发性病变。组织学表现由小而一致的神经元和脂肪样细胞组成。1例肿瘤细胞具有异型性,局部核分裂象易见,诊断为WHO2~3级;2例肿瘤细胞异型性明显,可见血管内皮细胞增生及小灶状坏死,呈非典型脂肪神经细胞瘤形态,诊断为WHO3级。免疫组化结果显示,9例肿瘤细胞均弥漫表达CD56、突触素(Syn)和神经元特异性烯醇化酶(neuron specific enolase,NSE),8例肿瘤细胞局灶表达巢蛋白(Nestin),5例肿瘤细胞局灶或弥漫表达胶质纤维酸性蛋白(glialfibrillaryacidic protein,GFAP),2例肿瘤细胞局灶表达S-100蛋白,3例个别肿瘤细胞少突胶质细胞系转录因子-2(Olig-2)阳性;肿瘤细胞均不表达神经元核抗原(NeuN)、神经丝(neurofilament,NF)和上皮细胞膜抗原(epithelial membrane antigen,EMA)。6例Ki-67增殖指数较低,为1%~4%,3例肿瘤细胞生长活跃的区域及呈非典型脂肪神经细胞瘤结构,Ki-67增殖指数为5%~20%。7例随访6~54个月,2例术后复发。结论脂肪神经细胞瘤是一种罕见的中枢神经系统肿瘤,好发于小脑半球,确诊需结合组织病理学特点和免疫组化结果,需与中枢神经细胞瘤、脑室外神经细胞瘤及少突胶质细胞瘤鉴别。首要治疗方式为手术切除,患者预后良好。
Objective To explore the clinicopathological characteristics,diagnosis and differential diagnosis of liponeurocytoma.Methods Nine patients diagnosed with liponeurocytoma in the Department of Pathology,Xuanwu Hospital,Capital Medical University and the Department of Pathology,the First Affiliated Hospital,Fujian Medical University from June 2011 to March 2020 were selected.The clinical data,imaging characteristics,pathological characteristics,immunophenotypes and prognosis of the patients were retrospectively analyzed.Results Among the nine patients,there were four males and five females,aged from 5 to 63 years old.The first symptoms were headache,dizziness with nausea and vomiting in eight cases,and walking instability in one case.The imaging showed that seven cases were located in the cerebellum hemisphere and one leision in lateral ventricle,all of which were single mass.Another case was multiple lesions located in lateral ventricle and medulla oblongata.The histological presentations consisted of small,uniform neurons and adipose cells.The tumor cells of one case showed cellular atypia and obvious local mitosis,which was diagnosed as WHO grade 2 to 3.In two cases,the tumor cells had obivous cellular atypia,including vascular endothelial cell proliferation and small focal necrosis,presenting atypical liposoneurocytoma morphology,which was diagnosed as WHO grade 3.Immunohistochemical results showed that CD56,synaptophysin(Syn)and neuron specific enolase(NSE)were diffusely expressed in tumor cells of nine cases.Nestin was focally expressed in tumor cells of eight cases.Glial fibrillary acidic protein(GFAP)was focally or diffusely expressed in tumor cells of five cases.S-100 protein was focally expressed in tumor cells of two cases.And Olig-2 was positive in individually tumor cells of three cases.NeuN,neurofilament(NF)and epithelial membrane antigen(EMA)were not expressed in tumor cells.The Ki-67 proliferative index of six patients was low(1%-4%),and which was 5%-20%in the area of active tumor cell growth and sho
作者
连芳
王丹丹
郑莉梅
赵莉红
王伟民
胡承聪
卢德宏
朴月善
Lian Fang;Wang Dandan;Zheng Limei;Zhao Lihong;Wang Weiming;Hu Chengcong;Lu Dehong;Piao Yueshan(Department of Pathology,Xuanwu Hospital,Capital Medical University,Beijing 100053,China)
出处
《北京医学》
CAS
2022年第5期403-406,411,共5页
Beijing Medical Journal
