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作 者:黄佛宝 刘泽斯 叶景 张良 林建浩 Huang Fobao;Liu Zesi;Ye Jing;Zhang Liang;Lin Jianhao(Department of Neurosurgery,Guangdong Sanjiu Brain Hospital,Guangzhou,Guangdong 510000,China)
机构地区:[1]广东三九脑科医院神经外科,广东广州510000
出 处:《中国微侵袭神经外科杂志》2024年第6期359-363,共5页Chinese Journal of Minimally Invasive Neurosurgery
摘 要:目的分析脑室外神经细胞瘤(extraventricular neurocytoma,EVN)的临床影像与病理特征。方法回顾性分析7例经术后病理证实为EVN病例的临床表现、影像学及组织病理学特征。结果7例患者的临床表现以颅高压症状及神经功能障碍为主,MRI呈长T1、等长T2,FLAIR呈高信号,DWI多为等高信号,呈轻中度不均匀强化,伴囊变、钙化等。免疫组化示所有病例突触素均阳性,3例胶质纤维酸性蛋白(glial fibrillary acidic protein,GFAP)阳性,4例少突胶质细胞转录因子2(oligodendrocyte transcription factor 2,Olig 2)阳性,2例神经元核特异蛋白(neuro-specific nuclei protein,Neu-N)阳性,4例Ki-67为1%~3%,3例Ki-67≥10%,最高达20%,5例异柠檬酸脱氢酶-1(isocitrate dehydrogenase enzyme isoform 1,IDH-1)阴性。4例病理诊断为EVN(WHOⅡ级),3例为非典型EVN(WHOⅡ~Ⅲ级)。肿瘤全切除6例,部分切除1例,无手术死亡病例,5例行术后辅助放疗。术后随访12~48个月,仅1例患者术后2年因肿瘤复发,再次行活检及放化疗外,余患者预后良好。结论EVN临床表现及影像学有一定特征,但易误诊,确诊需病理检查。手术全切除肿瘤是理想治疗方式,术后辅助放疗对部分患者是必要治疗措施。Objective To analyze the clinical imaging and pathological features of extraventricular neurocytoma(EVN).Methods The clinical manifestations,imaging,and histopathological features of 7 patients with EVN which was confirmed by postoperative pathology,were analyzed retrospectively.Results The clinical presentations of the 7 patients were primarily characterized by intracranial hypertension symptoms and neurological dysfunction.MRI revealed long T1,iso-T2 signals,high signals on FLAIR,and mostly iso-high signals on DWI,with mild to moderate heterogeneous enhancement,accompanied by cystic changes and calcifications.Immunohistochemical analysis showed positive synaptophysin in all the patients,glial fibrillary acidic protein(GFAP)positivity in 3,oligodendrocyte transcription factor 2(Olig-2)positivity in 4,neuro-specific nuclei protein(Neu-N)positivity in 2,Ki-67 labeling index ranging from 1%-3%in 4 and≥10%in 3(up to 20%),and isocitrate dehydrogenase enzyme isoform 1(IDH-1)negativity in 5.Pathological diagnoses included 4 cases of EVN(WHO gradeⅡ)and 3 of atypical EVN(WHO gradeⅡ-Ⅲ).Total tumor resection was achieved in 6 patients,with subtotal resection in 1 patient.There were no operative deaths,and 5 patients received postoperative adjuvant radiotherapy.During the postoperative follow-up period of 12 to 48 months,except for one patient who underwent re-biopsy and radiochemotherapy due to tumor recurrence,the remaining patients had good prognosis.Conclusions EVN exhibits certain clinical and imaging features but is prone to misdiagnosis,necessitating pathological examination for confirmation.Complete surgical resection is the ideal treatment,and postoperative adjuvant radiotherapy is necessary for some patients.
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