儿童室管膜瘤19例临床病理分析

Ependymomas in children: a clinical and pathological analysis of 19 cases

目的 探讨儿童室管膜瘤的临床病理学特点。方法 收集19例发生于儿童的室管膜瘤，根据临床病理学特点，形态学观察及免疫组化染色进行分析。结果 19例儿童室管膜瘤患者中男性12例，女性7例，年龄2-15岁，平均7．9岁。位于幕下者13例，位于幕上者5例，其中4例位于脑室外的大脑实质；发生于脊髓者仅1例。根据WHO分级Ⅰ级1例、Ⅱ级15例、Ⅲ级3例。最常见的病理表现为血管周围假菊形团形成，瘤细胞放射状排列在血管周围，形成血管周围无细胞核区。部分病例还可见室管膜裂隙和室管膜菊形团。9例进行了免疫组化染色，结果 显示大部分瘤细胞GFAP、S-100和vimentin（＋），部分瘤细胞EMA（＋），阳性信号主要分布于室管膜菊形团的腔面，呈线、点状分布。结论 室管膜瘤是儿童颅内肿瘤中常见的肿瘤之一，其最主要的组织病理学特点为血管周围形成假菊形团和室管膜菊形团。

Objective To analyse the clinical and pathological features of ependymoma in child so as to improve management of this disease. Methods The clinical and pathological characteristics of 19 cases of ependymomas in children were studied retrospectively. The main methods were paraffin-embedded sections histochemical staining and imrnunohistochemical staining （EnVisionTM method）. Results 12 cases were male and 7 cases were female among 19 cases. The patients＇ average age at diagnosis were 7.9 （2 - 15） years. The percentage of infratentorial ependymomoas and supratentorial ependymomas were 68.4% and 26.3 % respectively. 4 cases of supratentorial parenchymal ependymomas were located outside the ventricular system. Only 1 case （6.7%） occurred in spinal cord. The number of tumors according to WHO grade was 1 case of WHO grade Ⅰ and 15 cases of grade Ⅱ and 3 cases of grade Ⅲ respectively. The most frequently histological features were perivascular pseudorosettes. The perivascular pseudorosettes originated from tumour cells arranged radially around blood vessels. Ependymal rosettes could be seen in some cases. 9 cases were conducted immunohistochemical staining and the majority displayed GFAP and S-100 and vimentin immunoreactivity. Epithelial membrane antigen （EMA） immunoreactivity was showed in some tumour cells with a prominent signal along the lumen surface of ependymal rosettes. Conclusions Ependymoma is one of the turnouts occurred frequently in children. The most important histopathological features are perivascular pseudorosettes and ependymal rosettes, and the clinicalpathological features are essential for its diagnosis.