脑肿瘤1 Neoplasms of the Posterior 课件.pptVIP

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 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 Dysplastic Cerebellar Gangliocytoma commonly symptoms increased intracranial pressure hydrocephalus Megalencephaly mental retardation duration of symptoms considerable variability asymptomatic Dysplastic Cerebellar GangliocytomaVSCowden disease Dysplastic Cerebellar Gangliocytoma disruption of the normal cerebellar laminar structure hypertrophic ganglion cells granular and molecular layers of the cerebellar cortex increased myelination in the molecular layer Mitotic activity and necrosis are uncommon Dysplastic Cerebellar Gangliocytoma Dysplastic Cerebellar Gangliocytoma Dysplastic Cerebellar Gangliocytoma NCCT usually hypoattenuated may be isoattenuated Calcification is uncommon Thinning of the skull Dysplastic Cerebellar Gangliocytoma 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 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 Dysplastic Cerebellar Gangliocytoma Dysplastic Cerebellar Gangliocytoma Dysplastic Cerebellar Gangliocytoma Dysplastic Cerebellar Gangliocytoma Dysplastic Cerebellar Gangliocytoma Dysplastic Cerebellar GangliocytomaGoal of therapy Decompression of the ventricular system 解除脑室系统的压迫 difficult visualization: gradual change from normal cerebellar tissue to the abnormal tissue 正常脑组织与瘤组织分界不清 impairs a complete resection 难以完整