成人后颅窝脑肿瘤PPT.ppt

1/64 Hemangioblastoma 血管母细胞瘤，血管网状细胞瘤（血网） Dysplastic Cerebellar Gangliocytoma 发育不良性神经节细胞瘤 Subependymoma 室管膜下瘤 Adult Tumorsof the Posterior Fossa 2/64 3/64 Incidence and Clinical Presentation Dysplastic Cerebellar Gangliocytoma Originally described in 1920 also called Lhermitte-Duclos disease (LDD) neoplastic histogenesis Vs. hamartomatous origin young adults: average age 34 years no gender predilection 1920年首次报道 又称为LDD 早期研究认为其为肿瘤性病变，现认为其为错构瘤样改变 常见于年轻人，中位发病年龄34岁 无性别差异 4/64 Incidence and Clinical Presentation Dysplastic Cerebellar Gangliocytoma commonly symptoms increased intracranial pressure hydrocephalus Megalencephaly mental retardation duration of symptoms considerable variability asymptomatic 常见临床症状 颅内压增高 脑积水 巨脑畸形 精神障碍 症状时间 不定 甚至也可无症状 5/64 Dysplastic Cerebellar GangliocytomaVSCowden disease Cowden disease autosomal dominant hamartoma syndrome characterized syndrome mucocutaneous lesions macrocephaly hamartomas long arm of chromosome 10 Cowden病 常染色体显性遗传 错构瘤样病变 特征性表现 皮肤粘膜病变 巨脑畸形 错构瘤 10号染色体长臂异常 Clinical Presentation 8/64 Pathologic Findings Dysplastic Cerebellar Gangliocytoma 免疫组化染色显示神经元特异性蛋白酶及突触素阳性表达 9/64 Imaging Findings-CT NCCT usually hypoattenuated may be isoattenuated Calcification is uncommon Thinning of the skull Dysplastic Cerebellar Gangliocytoma CT平扫 常为低密度 但也可为等密度 无特异性 诊断困难 钙化少见 偶可见颅板变薄 10/64 Imaging Findings-MRI MRI：best imaging modality characteristic appearance without histopathological confirmation typical appearances 1. unilateral cerebellar mass 2. non-enhancing 3. middle-aged patient 4. tiger-striped pattern Dysplastic Cerebellar Gangliocytoma MRI是诊断的最佳方式 特征性表现 几乎可在不需要病理证实的条件下做出明确诊断 四条特征 1. 单侧半球肿块 2. 不强化 3. 成年患者 4. 虎斑征 11/64 Imaging Findings-MRI characteristic: bands hyperintensity and isointensity on T2 isointense and hypointense on T1 hyperintense signal on T2 corresponds to the inner molecular layer, granular cell layer, and loss of central white matter Dysplastic Cerebellar Gangliocytoma 特征性表现：条带 T2等、高信号 T1等低信号 T2上所见的高信号条带为内分子层、颗粒细胞层，以