室管膜下瘤的临床特征和预后

Clinical Features and Prognosis of Subependymoma

目的探讨室管膜下瘤的临床特征及预后。方法收集2012年1月1日至2021年9月30日郑州大学第一附属医院收治的26例室管膜下瘤患者。回顾性分析其临床表现、影像、病理特点、治疗及预后。结果在26例室管膜下瘤患者中,男15例,女11例,男女之比约为1.3∶1;发病年龄27-66岁,中位年龄44岁。24例(92.3%)肿瘤位于颅内(22例位于脑室,2例位于脑实质),余2例位于颈髓。颅内肿瘤最大径为2.1-8.1cm,平均3.4cm;颈髓内为长条形肿块,长径分别为6.1cm和8.0cm。磁共振成像(MRI)呈团块状影,61.5%(16/26)的肿瘤界限清,多为长T_(1)长T_(2)信号(75.0%,12/16)。患者的主要临床症状为头痛(65.4%,17/26)、头晕(30.4%,7/23),可伴视物不清或视力下降、恶心呕吐。26例均为手术切除标本,大体灰白、灰红色,切面灰白,质软至中,显微镜下低倍多呈分叶状,高倍主要表现为丰富胶质纤维基质背景中见簇状分布的细胞核,部分呈束状流水状排列,常伴微囊形成,细胞核小至中等大小,圆形或卵圆形,形态一致,核分裂罕见。免疫组织化学结果显示:肿瘤细胞表达GFAP(100.0%,20/20),S-100蛋白(100.0%,20/20),部分表达NSE(66.7%,2/3),不表达EMA、Oligo-2、Syn、Neu-N、IDH1和AE1/AE3,肿瘤细胞Ki-67增殖指数低(<5%)。临床神经外科医生均采用手术完整切除肿瘤。26例患者中18例获得随访结果,其中17例存活。结论室管膜下瘤是一种罕见但预后好的中枢神经系统肿瘤,具有典型的MRI及组织学改变,结合免疫组化易于诊断并与其他低级别胶质肿瘤鉴别。

Objective To investigate the clinical features and prognosis of subependymoma.Methods Twenty-six patients with subependymoma admitted to the First Affliated Hospital of Zhenghou University from January 1,2012 to September 30,2021 were collected.The clinical manifestations,imaging,pathological features,treatment and prognosis were retrospectively analyzed.Results Among the 26 patients with subependymoma,there were 15 males and 11 females,with a male-to-female ratio of about 1.3 to 1.The onset age ranged from 27 to 66 years old,with a median age of 44 years.Twenty-four cases(92.3%)of tumors were located in the brain(22 cases were located in the brain ventricle and 2 cases were located in the brain parenchyma),and the remaining 2 cases were located in the cervical spinal cord.The maximum diameter of intracranial tumors was 2.1-8.1 cm,and the average maximum diameter was 3.4 cm.The cervical spinal cord was a long-striped mass with a length of6.1 cm and 8.0 cm,respectively.Magnetic resonance imaging(MRI)showed mass shadows,and 61.5%(16/26)of the tumors had clear boundaries,and most of them had long T_(1) and long T_(2) signals(75.0%,12/16).The main clinical symptoms were headache(65.4%,17/26),dizziness(30.4%,7/23),which could be accompanied by blurred vision or decreased vision,nausea and vomiting.All 26 cases were surgically resected specimens,generally gray-white,gray-red,gray-white in section,soft to medium,mostly lobulated at low magnification,and clustered nuclei in the background of rich glial fibrous matrix at high magnifcation.Some of them were arranged in fascicles,and often accompanied by the formation o microcapsules.The muclei were small to medium in size,round or oval,consistent in shape,and mitoses were rare.Immunohistochemical results showed that tumor ellls expressed GFAP(100.0%,20/20),S-100 protein(100.0%,20/20),partially expressed NSE(66.7%,2/3),but did not express EMA,Oligo-2,Syn,Neu-N,IDH1 and AE1/AE3.The Ki-67 proliferation index of tumor cells was low(<5%).Clinical neurosurgeons all used surgery to completely remove the tumor.Follow-up results were obtained in 18 of the 26 patients,and 17 patients were alive.Conclusion Subependymoma is a rare but good prognosis central nervous system tumor with typical MRI and histological changes.Combined with immunohistochemistry,it can be easily diagnosed and differentiated from other low-grade gliomas.