少见的脑室内节细胞胶质瘤2例临床病理观察

Intraventricular gangliogliomas:a clinicopathological analysis of two rare cases

目的探讨脑室内节细胞胶质瘤(GG)的病理诊断、鉴别诊断及预后。方法收集了2例脑室内GG,并结合国内已报道病例分析临床特点,组织学形态及预后。结果患者临床上主要症状为头痛、呕吐,其次为视力减退及癫。镜下肿瘤细胞形态多样,由肿瘤性胶质细胞(毛细胞性星形细胞瘤或少突胶质细胞瘤样成分)及发育不良的节细胞组成,可见Rosenthal纤维及嗜酸性颗粒小体,伴少许钙化及血管周淋巴细胞袖套形成。结论脑室内GG较为少见,多为WHOⅠ级肿瘤,手术切除后预后较好。因该肿瘤少见,镜下形态多样,易与多种肿瘤混淆,根据其胶质神经元组织学特征并进行相应的免疫组化检测有助于鉴别诊断,准确诊断对治疗及预后具有重要意义。

Objective To explore the pathological diagnosis,differential diagnosis and prognosis of intraventricular gangliogliomas（ GG-V）.Methods A total of 11 cases with intraventricular ganglioglioma were diagnosed by the surgical pathology examination from the department of pathology of PLA general hospital with review of the relevant literature.The11 cases included 8 males and 3 females,with a mean age of 24.4.The headache,vomiting,decreased visual acuity and epilepsy were found in 11 cases.Tumors were located in the lateral ventricle in 6 cases,the third ventricle in 2 cases,the lateral ventricle extended into the third and fourth ventricle in 2 cases,genu of corpus callosum and septum pellucidum in 1case.Tumor was studied by paraffin slices,HE staining and immunohistochemistry.Results Under microscope,it was revealed a biphasic histological architecture characterized by a combination of neoplastic glial cell and dysplastic neurons elements.While rosenthal fibers or eosinophilic granular bodies in the lesion and perivascular lymphocyte cuffing were also the characterized findings.One case was recurrence after twelve months of operation.Conclusions Intraventricular gangliogliomas（ GG） is extremely rare.GG is a low-grade tumor corresponding to WHO grade Ⅰwith a good prognosis after total resection.GG is prone to confuse with other tumors caused by rareness and morphological diversity under microscope.GG was usually final diagnosis by histopathological and immunohistochemical features.The accurate diagnosis play an important role in patient＇s therapy and prognosis.