成人后颅窝脑肿瘤课件_1

1/64 HemangioblastomaHemangioblastoma l l 血管母细胞瘤，血管网状细胞瘤（血网）血管母细胞瘤，血管网状细胞瘤（血网） Dysplastic Cerebellar GangliocytomaDysplastic Cerebellar Gangliocytoma l l 发育不良性神经节细胞瘤发育不良性神经节细胞瘤 SubependymomaSubependymoma l l 室管膜下瘤室管膜下瘤 Adult TumorsAdult Tumors of the Posterior Fossaof the Posterior Fossa 2/64 3/64 Incidence and Clinical Presentation Dysplastic Cerebellar GangliocytomaDysplastic Cerebellar Gangliocytoma Originally described in Originally described in 19201920 also called Lhermitte-also called Lhermitte- Duclos disease (LDD)Duclos disease (LDD) neoplastic histogenesis neoplastic histogenesis Vs. hamartomatous Vs. hamartomatous originorigin young adults: average young adults: average age 34 yearsage 34 years no gender predilectionno gender predilection 19201920年首次报道年首次报道 又称为又称为LDDLDD 早期研究认为其为肿瘤性早期研究认为其为肿瘤性 病变，现认为其为错构瘤病变，现认为其为错构瘤 样改变样改变 常见于年轻人，中位发病常见于年轻人，中位发病 年龄年龄3434岁岁 无性别差异无性别差异 4/64 Incidence and Clinical Presentation Dysplastic Cerebellar GangliocytomaDysplastic Cerebellar Gangliocytoma commonly symptomscommonly symptoms l l increased intracranial increased intracranial pressurepressure l l hydrocephalushydrocephalus l l MegalencephalyMegalencephaly l l mental retardationmental retardation duration of symptomsduration of symptoms l l considerable variabilityconsiderable variability asymptomaticasymptomatic 常见临床症状常见临床症状 l l 颅内压增高颅内压增高 l l 脑积水脑积水 l l 巨脑畸形巨脑畸形 l l 精神障碍精神障碍 症状时间症状时间 l l 不定不定 甚至也可无症状甚至也可无症状 5/64 Dysplastic Cerebellar GangliocytomaDysplastic Cerebellar Gangliocytoma VSVS Cowden diseaseCowden disease Cowden diseaseCowden disease l l autosomal dominantautosomal dominant l l hamartoma syndromehamartoma syndrome characterized syndrome characterized syndrome l l mucocutaneous lesionsmucocutaneous lesions l l macrocephalymacrocephaly l l hamartomashamartomas long arm of chromosome long arm of chromosome 1010 CowdenCowden病病 l l 常染色体显性遗传常染色体显性遗传 l l 错构瘤样病变错构瘤样病变 特征性表现特征性表现 l l 皮肤粘膜病变皮肤粘膜病变 l l 巨脑畸形巨脑畸形 l l 错构瘤错构瘤 1010号染色体长臂异常号染色体长臂异常 Clinical Presentation 8/64 Pathologic Findings Dysplastic Cerebellar GangliocytomaDysplastic Cerebellar Gangliocytoma 免疫组化染色显示神经元免疫组化染色显示神经元 特异性蛋白酶及突触素阳特异性蛋白酶及突触素阳 性表达性表达 9/64 Imaging Findings-CT NCCTNCCT l l usually hypoattenuatedusually hypoattenuated l l may be isoattenuatedmay be isoattenuated l l Calcification is Calcification is uncommonuncommon l l Thinning of the skullThinning of the skull Dysplastic Cerebellar GangliocytomaDysplastic Cerebellar Gangliocytoma CTCT平扫平扫 l l 常为低密度常为低密度 l l 但也可为等密度但也可为等密度 l l 无特异性无特异性 l l 诊断困难诊断困难 l l 钙化少见钙化少见 l l 偶可见颅板变薄偶可见颅板变薄 10/64 Imaging Findings-MRI MRIMRI：best imaging modalitybest imaging modality l l characteristic appearancecharacteristic appearance l l without histopathological without histopathological confirmationconfirmation typical appearancestypical appearances l l 1. unilateral cerebellar mass1. unilateral cerebellar mass l l 2. non-enhancing2. non-enhancing l l 3. middle-aged patient3. middle-aged patient l l 4. tiger-striped pattern4. tiger-striped pattern Dysplastic Cerebellar GangliocytomaDysplastic Cerebellar Gangliocytoma MRIMRI是诊断的最佳方式是诊断的最佳方式 l l 特征性表现特征性表现 l l 几乎可在不需要病理证实几乎可在不需要病理证实 的条件下做出明确诊断的条件下做出明确诊断 四条特征四条特征 l l 1. 1. 单侧半球肿块单侧半球肿块 l l 2. 2. 不强化不强化 l l 3. 3. 成年患者成年患者 l l 4. 4. 虎斑征虎斑征 11/64 Imaging Findings-MRI characteristic: bandscharacteristic: bands l l hyperintensity and hyperintensity and isointensity on T2isointensity on T2 l l isointense and hypointense isointense and hypointense on T1on T1 l l hyperintense signal on T2 hyperintense signal on T2 corresponds to the inner corresponds to the inner molecular layer, granular cell molecular layer, granular cell layer, and loss of central layer, and loss of central white matterwhite matter Dysplastic Cerebellar GangliocytomaDysplastic Cerebellar Gangliocytoma 特征性表现：条带特征性表现：条带 l l T2T2等、高信号等、高信号 l l T1T1等低信号等低信号 l l T2T2上所见的高信号条上所见的高信号条 带为内分子层、颗粒带为内分子层、颗粒 细胞层，以及白质细细胞层，以及白质细 胞丢失所致胞丢失所致 12/64 Imaging Findings-MRI Dysplastic Cerebellar GangliocytomaDysplastic Cerebellar Gangliocytoma Dysplastic cerebellar gangliocytoma in a 47-year-old woman. (a)Axial T1-Dysplastic cerebellar gangliocytoma in a 47-year-old woman. (a)Axial T1- weighted MR image shows a cerebellar mass with a striped appearance. weighted MR image shows a cerebellar mass with a striped appearance. (b)Axial T2-weighted MR image shows the same laminar morphology, (b)Axial T2-weighted MR image shows the same laminar morphology, composed of alternating hyperintense and isointense bands. (c) Con-trast-composed of alternating hyperintense and isointense bands. (c) Con-trast- enhanced axial T1-weighted MR image shows no enhancement of the mass.enhanced axial T1-weighted MR image shows no enhancement of the mass. 13/64 Imaging Findings-MRI Dysplastic Cerebellar GangliocytomaDysplastic Cerebellar Gangliocytoma 虎斑征，条纹征虎斑征，条纹征 14/64 Imaging Findings-MRI Dysplastic Cerebellar GangliocytomaDysplastic Cerebellar Gangliocytoma 15/64 Imaging Findings-MRI Dysplastic Cerebellar GangliocytomaDysplastic Cerebellar Gangliocytoma 1.5T SWI MIP1.5T SWI MIP图像显示瘤周引流静脉，图像显示瘤周引流静脉，7T SWI MIP7T SWI MIP图像显示瘤图像显示瘤 周大量引流静脉及齿状核受压周大量引流静脉及齿状核受压 16/64 Imaging Findings-MRI Dysplastic Cerebellar GangliocytomaDysplastic Cerebellar Gangliocytoma 图图1 1 MRIMRI平扫轴位平扫轴位T1WI(A)T1WI(A)，增强扫描轴位，增强扫描轴位T1WI(B)T1WI(B)，左侧小脑半球可见一团，左侧小脑半球可见一团 块状长块状长T1T1、长、长T2T2异常信号影，边界清楚，其内信号不均匀，可见条纹状等异常信号影，边界清楚，其内信号不均匀，可见条纹状等T1T1、 T2T2信号影。增强扫描未见明显强化。图信号影。增强扫描未见明显强化。图2 2 各向异性分数（各向异性分数（fractional fractional anisotropic, FAanisotropic, FA）灰度图，肿瘤区域呈低信号。图灰度图，肿瘤区域呈低信号。图3 ADC3 ADC图，肿瘤区域呈等及稍图，肿瘤区域呈等及稍 高信号高信号 17/64 Imaging Findings-MRI Dysplastic Cerebellar GangliocytomaDysplastic Cerebellar Gangliocytoma 各向异性分数（各向异性分数（fractional anisotropic, FAfractional anisotropic, FA）方向彩色编码）方向彩色编码 图上正常小脑区域呈绿色图上正常小脑区域呈绿色( (表示前后走行表示前后走行) )，肿瘤区以红色，肿瘤区以红色 为主为主( (表示左右走行表示左右走行) )，未见条样信号。图，未见条样信号。图5 5 纤维束追踪成 纤维束追踪成 像像像像(Fiber tractography(Fiber tractography）。）。fiber tract within the tumorfiber tract within the tumor肿肿 瘤区纤维束以左右方向走行为主。瘤区纤维束以左右方向走行为主。 18/64 Dysplastic Cerebellar GangliocytomaDysplastic Cerebellar Gangliocytoma Goal of therapyGoal of therapy Decompression of the ventricular systemDecompression of the ventricular system 解除脑室系统的压迫解除脑室系统的压迫 difficult visualization: gradual change from normal difficult visualization: gradual change from normal cerebellar tissue to the abnormal tissuecerebellar tissue to the abnormal tissue 正常脑组织与瘤组织分界不清正常脑组织与瘤组织分界不清 impairs a complete resectionimpairs a complete resection 难以完整切除难以完整切除 most patients do well following surgical resectionmost patients do well following surgical resection 大多数患者预后良好大多数患者预后良好 some have recurrence after a prolonged disease-free some have recurrence after a prolonged disease-free intervalinterval 少数患者经过一段静止期后仍可复发少数患者经过一段静止期后仍可复发 19/64 HemangioblastomaHemangioblastoma l l 血管母细胞瘤，血管网状细胞瘤（血网）血管母细胞瘤，血管网状细胞瘤（血网） Dysplastic Cerebellar GangliocytomaDysplastic Cerebellar Gangliocytoma l l 发育不良性神经节细胞瘤发育不良性神经节细胞瘤 SubependymomaSubependymoma l l 室管膜下瘤室管膜下瘤 PART TWOPART TWO Adult TumorsAdult Tumors of the Posterior Fossaof the Posterior Fossa 20/64 SUMMARYSUMMARY Often Occurs in middle-aged menOften Occurs in middle-aged men 好发于中年男性好发于中年男性 fourth ventricle and lateral ventriclefourth ventricle and lateral ventricle 多位于四脑室和侧脑室多位于四脑室和侧脑室 Mass, clearly edgeMass, clearly edge 团块状、边缘较清楚团块状、边缘较清楚 no or slight Enhancedno or slight Enhanced 增强扫描后，多无强化或轻微强化增强扫描后，多无强化或轻微强化 21/64 Incidence and Clinical Presentation SubependymomaSubependymoma 19451945年由年由ScheinkerScheinker首次报道首次报道 占颅内全部肿瘤的不足占颅内全部肿瘤的不足1%1% 大多数学者认为室管膜下瘤是室管膜瘤的一种类型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、室管膜下巨细胞星室管膜下瘤、中枢神经细胞瘤、室管膜下巨细胞星 形细胞瘤形细胞瘤SubependymomaSubependymoma, central neurocytoma, , central neurocytoma, subependymal giant cell astrocytomasubependymal giant cell astrocytoma具有相同的起源具有相同的起源 ，均起源于室管膜下的具有双向分化潜能的神经胶，均起源于室管膜下的具有双向分化潜能的神经胶 质祖细胞质祖细胞Glial progenitor cellsGlial progenitor cells 22/64 Incidence and Clinical Presentation SubependymomaSubependymoma 23/64 Incidence and Clinical Presentation SubependymomaSubependymoma rare, benign, slowgrowing tumor rare, benign, slowgrowing tumor generally well-circumscribedgenerally well-circumscribed sometimes multiple lesionssometimes multiple lesions most frequently: fourth ventriclemost frequently: fourth ventricle distinctive histologic appearancedistinctive histologic appearance histogenesis: controversialhistogenesis: controversial 良性肿瘤，生长缓慢良性肿瘤，生长缓慢 通常边界清楚通常边界清楚 可为多发可为多发 常见于四脑室常见于四脑室 病理学表现典型病理学表现典型 组织学发生有争议组织学发生有争议 24/64 Incidence and Clinical Presentation SubependymomaSubependymoma arises from: subependymal arises from: subependymal glial layerglial layer asymptomatic, incidentally asymptomatic, incidentally at autopsy (0.4%)at autopsy (0.4%) Males more commonlyMales more commonly Most cases (82%) older Most cases (82%) older than 15 yearsthan 15 years 起源于室管膜下起源于室管膜下 通常无症状通常无症状 常常在尸检中发现常常在尸检中发现 男性多见男性多见 大多数为大多数为1515岁以上成人岁以上成人 25/64 Incidence and Clinical Presentation SubependymomaSubependymoma half of the reported half of the reported casescases：fourth ventriclefourth ventricle 404045% lateral 45% lateral ventricleventricle septum pellucidumseptum pellucidum third ventriclethird ventricle spinal cordspinal cord total surgical resectiontotal surgical resection Recurrence rareRecurrence rare 半数以上病例见于四脑室半数以上病例见于四脑室 其余大部分位于侧脑室其余大部分位于侧脑室 少见部位少见部位 l l 透明隔透明隔 l l 三脑室三脑室 l l 脊髓脊髓 手术切除是治疗方法手术切除是治疗方法 复发少见复发少见 26/64 Incidence and Clinical Presentation SubependymomaSubependymoma clinical presentation: clinical presentation: non-specificnon-specific location, size, and location, size, and intratumoral intratumoral hemorrhagehemorrhage hydrocephalushydrocephalus neurologic deficits, neurologic deficits, seizures, SAHseizures, SAH good prognosisgood prognosis 临床症状不典型临床症状不典型 临床症状取决于肿瘤的部临床症状取决于肿瘤的部 位、大小、是否合并有肿位、大小、是否合并有肿 瘤内出血等瘤内出血等 常见症状为脑积水所致的常见症状为脑积水所致的 症状症状 其它也可有神经功能缺损其它也可有神经功能缺损 、癫痫、蛛网膜下腔出血、癫痫、蛛网膜下腔出血 等等 手术后预后良好手术后预后良好 27/64 Pathologic Findings GrosslyGrossly l l well-circumscribed mass well-circumscribed mass l l firm texturefirm texture l l white to grayish colorwhite to grayish color l l narrow pediclenarrow pedicle l l grows slowgrows slow l l avascularavascular l l smaller than 2cm in smaller than 2cm in diameterdiameter SubependymomaSubependymoma 大体观大体观 l l 边界清晰边界清晰 l l 质地坚实质地坚实 l l 灰白相间灰白相间 l l 有窄蒂有窄蒂 l l 生长缓慢生长缓慢 l l 乏血供乏血供 l l 直径通常小于直径通常小于2 2厘米厘米 28/64 Pathologic Findings HistologicHistologic l l dense fibrillary matrixdense fibrillary matrix l l numerous small cystsnumerous small cysts l l isomorphic nucleiisomorphic nuclei l l Mitotic: lowMitotic: low l l WHO grade IWHO grade I l l Admixture with Admixture with ependymomaependymoma SubependymomaSubependymoma 组织病理学组织病理学 l l 致密纤维基质致密纤维基质 l l 大量小囊大量小囊 l l 同构核同构核 l l 有丝分裂少见有丝分裂少见 l l WHO IWHO I级级 l l 可与室管膜瘤混合生长可与室管膜瘤混合生长 29/64 Pathologic Findings SubependymomaSubependymoma HE staining Immunohistochemistry HE staining Immunohistochemistry 30/64 Imaging Findings SubependymomaSubependymoma CTCT l l well-circumscribedwell-circumscribed l l lobulatedlobulated l l intraventricular massintraventricular mass l l hydrocephalushydrocephalus l l hypoattenuatedhypoattenuated l l Calcification: commonCalcification: common l l cystic degeneration: cystic degeneration: commoncommon l l most (84%) enhancemost (84%) enhance CTCT图像上图像上 l l 边界清晰边界清晰 l l 有分叶有分叶 l l 脑室内肿块脑室内肿块 l l 脑积水脑积水 l l 低密度低密度 l l 钙化、囊性变多见钙化、囊性变多见 l l 增