幕上原始神经外胚叶瘤七例报告

Supratentorial Primitive Neuroectodermal Tumors

目的:探讨幕上原始神经外胚叶瘤(SPNET)的临床、影像和病理特征,以提高其诊断准确率。方法:对7例经手术及病理证实的SPNET的资料进行总结,重点分析影像学表现和组织病理学特征,并简述其临床表现、治疗及预后。结果:7例中5例发生在儿童,临床主要表现为颅内高压症状。CT和MRI检查,肿瘤均表现为幕上较大肿块,最大径4～10cm,其中4例有钙化灶,5例无瘤周水肿,增强检查7例均呈不均匀强化。病理检查示瘤细胞体积小,胞浆少,多呈裸核状,纤维芯菊形团结构常见;免疫组化染色示7例胶质纤维酸性蛋白均为阳性。结论:SPNET的影像学和组织病理学表现均有一定特征,影像学及临床表现相结合有助于术前与其他肿瘤鉴别,提高诊断准确性。

Objective： To investigate the clinical,imaging findings, and histopathologic features of supratentorial primitive neuroectodermal tumor （SPNET） for increasing the accuracy rate of diagnosis. Methods： The clinical materials of 7 SPNETs which included image findings,histopathologic features,diagnosis,differential diagnosis and treatment were summarized and analyzed. Results： Five cases happened in children. The tumors showed calcification in 4 cases and minimal peritumoral edema in 5 cases, All cases appeared heterogenous enhancement. The histopathologic features showed small cell size and high nucleolus/cytoplasm ratio. The structure of Homer-Wright rosette was commonly seen. GFAP was positive in all cases. Conclusion There are some characteristics on imaging and pathology of SPNET. It is helpful to differentiate SPENT from other tumors before operation by combining the imagines with clinical manifestation. This can increase the accuracy rate of diagnosis.