成人后颅窝脑肿瘤

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 1920also called Lhermitte-Duclos disease (LDD)neoplastic histogenesis Vs. hamartomatous originyoung adults: average age 34 yearsno 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 symptomsincreased intracranial pressurehydrocephalusMegalencephalymental retardationduration of symptomsconsiderable variabilityasymptomatic,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 diseaseautosomal dominanthamartoma syndromecharacterized syndrome mucocutaneous lesionsmacrocephalyhamartomaslong 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,6/64,Pathologic Findings,disruption of the normal cerebellar laminar structurehypertrophic ganglion cellsgranular and molecular layers of the cerebellar cortexincreased myelination in the molecular layerMitotic activity and necrosis are uncommon,Dysplastic Cerebellar Gangliocytoma,正常小脑板层结构破坏大量增生肥大的神经节细胞侵蚀小脑皮层的颗粒层和分子层分子层髓鞘化增加病理组织切片上有丝分裂活性及坏死少见,7/64,Pathologic Findings,Dysplastic Cerebellar Gangliocytoma,200,100,hypertrophic ganglion cells expanding the granular and molecular layers of the cerebellar cortex,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,NCCTusually hypoattenuatedmay be isoattenuatedCalcification is uncommonThinning 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 modalitycharacteristic appearancewithout histopathological confirmation typical appearances1. unilateral cerebellar mass2. non-enhancing3. middle-aged patient4. 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: bandshyperintensity and isointensity on T2isointense and hypointense on T1hyperintense 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，以及白质细胞丢失所致,12/64,Imaging Findings-MRI,Dysplastic Cerebellar Gangliocytoma,Dysplastic cerebellar gangliocytoma in a 47-year-old woman. (a)Axial T1-weighted MR image shows a cerebellar mass with a striped appearance. (b)Axial T2-weighted MR image shows the same laminar morphology, composed of alternating hyperintense and isointense bands. (c) Con-trast-enhanced axial T1-weighted MR image shows no enhancement of the mass.,13/64,Imaging Findings-MRI,Dysplastic Cerebellar Gangliocytoma,虎斑征，条纹征,14/64,Imaging Findings-MRI,Dysplastic Cerebellar Gangliocytoma,15/64,Imaging Findings-MRI,Dysplastic Cerebellar Gangliocytoma,1.5T SWI MIP图像显示瘤周引流静脉，7T SWI MIP图像显示瘤周大量引流静脉及齿状核受压,16/64,Imaging Findings-MRI,Dysplastic Cerebellar Gangliocytoma,图1MRI平扫轴位T1WI(A)，增强扫描轴位T1WI(B)，左侧小脑半球可见一团块状长T1、长T2异常信号影，边界清楚，其内信号不均匀，可见条纹状等T1、T2信号影。增强扫描未见明显强化。图2各向异性分数（fractional anisotropic, FA）灰度图，肿瘤区域呈低信号。图3 ADC图，肿瘤区域呈等及稍高信号,17/64,Imaging Findings-MRI,Dysplastic Cerebellar Gangliocytoma,各向异性分数（fractional anisotropic, FA）方向彩色编码图上正常小脑区域呈绿色(表示前后走行)，肿瘤区以红色为主(表示左右走行)，未见条样信号。图5纤维束追踪成像像(Fiber tractography）。fiber tract within the tumor肿瘤区纤维束以左右方向走行为主。,18/64,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难以完整切除most patients do well following surgical resection大多数患者预后良好some have recurrence after a prolonged disease-free interval少数患者经过一段静止期后仍可复发,19/64,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,PART TWOAdult Tumorsof the Posterior Fossa,20/64,SUMMARY,Often Occurs in middle-aged men好发于中年男性fourth ventricle and lateral ventricle多位于四脑室和侧脑室Mass, clearly edge团块状、边缘较清楚no or slight Enhanced增强扫描后，多无强化或轻微强化,21/64,Incidence and Clinical Presentation,Subependymoma,1945年由Scheinker首次报道占颅内全部肿瘤的不足1%大多数学者认为室管膜下瘤是室管膜瘤的一种类型室管膜下瘤、中枢神经细胞瘤、室管膜下巨细胞星形细胞瘤Subependymoma, central neurocytoma, subependymal giant cell astrocytoma具有相同的起源，均起源于室管膜下的具有双向分化潜能的神经胶质祖细胞Glial progenitor cells,22/64,Incidence and Clinical Presentation,Subependymoma,23/64,Incidence and Clinical Presentation,Subependymoma,rare, benign, slowgrowing tumor generally well-circumscribedsometimes multiple lesionsmost frequently: fourth ventricledistinctive histologic appearancehistogenesis: controversial,良性肿瘤，生长缓慢通常边界清楚可为多发常见于四脑室病理学表现典型组织学发生有争议,24/64,Incidence and Clinical Presentation,Subependymoma,arises from: subependymal glial layerasymptomatic, incidentally at autopsy (0.4%)Males more commonlyMost cases (82%) older than 15 years,起源于室管膜下通常无症状常常在尸检中发现男性多见大多数为15岁以上成人,25/64,Incidence and Clinical Presentation,Subependymoma,half of the reported cases：fourth ventricle4045% lateral ventricleseptum pellucidumthird ventriclespinal cordtotal surgical resectionRecurrence rare,半数以上病例见于四脑室其余大部分位于侧脑室少见部位透明隔三脑室脊髓手术切除是治疗方法复发少见,26/64,Incidence and Clinical Presentation,Subependymoma,clinical presentation: non-specificlocation, size, and intratumoral hemorrhagehydrocephalusneurologic deficits, seizures, SAHgood prognosis,临床症状不典型临床症状取决于肿瘤的部位、大小、是否合并有肿瘤内出血等常见症状为脑积水所致的症状其它也可有神经功能缺损、癫痫、蛛网膜下腔出血等手术后预后良好,27/64,Pathologic Findings,Grosslywell-circumscribed mass firm texturewhite to grayish colornarrow pediclegrows slowavascularsmaller than 2cm in diameter,Subependymoma,大体观边界清晰质地坚实灰白相间有窄蒂生长缓慢乏血供直径通常小于2厘米,28/64,Pathologic Findings,Histologicdense fibrillary matrixnumerous small cystsisomorphic nucleiMitotic: lowWHO grade IAdmixture with ependymoma,Subependymoma,组织病理学致密纤维基质大量小囊同构核有丝分裂少见WHO I级可与室管膜瘤混合生长,29/64,Pathologic Findings,Subependymoma,HE staining Immunohistochemistry,30/64,Imaging Findings,Subependymoma,CTwell-circumscribedlobulatedintraventricular masshydrocephalushypoattenuatedCalcification: commoncystic degeneration: commonmost (84%) enhance,CT图像上边界清晰有分叶脑室内肿块脑积水低密度钙化、囊性变多见增强后大多数强化,31