幕上原始神经外胚瘤五例

摘要

目的 探讨幕上原始神经外胚瘤(supratentorial primitive neuroectodermal tumor,SPNET)的临床表现、影像和病理特征.方法 对5例经病理证实的SPNET的资料,分析其临床表现、影像学组织病理学特征、治疗经过及预后.结果 5例中3例为儿童,2例为成人,主要临床表现为颅内高压症状.CT和MRI检查示肿瘤为幕上肿块,呈类圆形或分叶状,边界清楚,2例有钙化灶,3例无瘤周水肿.病理结果 提示肿瘤组织主要由小圆形细胞组成,部分为梭形细胞,细胞密集成巢,由纤维结缔组织分隔成小叶状,核染色质颗粒状,核仁不明显,多数可见核分裂象,形成Homer Wright菊形团.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相鉴别,提高诊断准确性.%Objective To discuss the clinical, imaging findings, and histopathologic features of supratentorial primitive neuroectodermal tumor ( SPNET ) supply data for treatment and diagnosis successfully. Methods The clinical materials of 5 SPNETs which included manifestations, imaging findings , histopathologic features diagnosis, treatment and prognosis were summarized and analyzed retrospectively. All the patients underwent microsurgery. Results Three cases happene.d in children and two in adults . The most manifestations were increased intracranial pressure .The tumors showed calcification in 2cases and minimal peritumoral edema in 3 cases. The lesions were round-like or lobulated in shape and well demarked. The histopathologic features showed fibrous bands surrounding nests of small round cell or spindle cell. Homer Wright rosettes could be found. There were granular nuclear chromatin and obscure nucleoli in the tumor cells. 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 and increase the accuracy rate of diagnosis.