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1、Hemangioblastoma血管母細(xì)胞瘤，血管網(wǎng)狀細(xì)胞瘤（血網(wǎng)）DysplasticCerebellarGangliocytoma發(fā)育不良性神經(jīng)節(jié)細(xì)胞瘤Subependymoma室管膜下瘤PARTTWOAdultTumorsofthePosteriorFossaIncidenceandClinicalPresentationDysplasticCerebellarGangliocytomaOriginallydescribedin1920alsocalledLhermitte-Duclosdisease(LDD)neoplastichistogenesisVs.hamartomatou
2、soriginyoungadults:averageage34yearsnogenderpredilection1920年首次報(bào)道又稱為LDD早期研究認(rèn)為其為腫瘤性病變，現(xiàn)認(rèn)為其為錯(cuò)構(gòu)瘤樣改變常見于年輕人，中位發(fā)病年齡34歲無性別差異IncidenceandClinicalPresentationDysplasticCerebellarGangliocytomacommonlysymptomsincreasedintracranialpressurehydrocephalusMegalencephalymentalretardationdurationofsymptomsconsidera
3、blevariabilityasymptomatic常見臨床癥狀顱內(nèi)壓增高腦積水巨腦畸形精神障礙癥狀時(shí)間不定甚至也可無癥狀DysplasticCerebellarGangliocytomaVS CowdendiseaseCowdendiseaseautosomaldominanthamartomasyndromecharacterizedsyndromemucocutaneouslesionsmacrocephalyhamartomaslongarmofchromosome10Cowden病常染色體顯性遺傳錯(cuò)構(gòu)瘤樣病變特征性表現(xiàn)皮膚粘膜病變巨腦畸形錯(cuò)構(gòu)瘤10號(hào)染色體長臂異常Clinical
4、PresentationPathologicFindingsdisruptionofthenormalcerebellarlaminarstructurehypertrophicganglioncellsgranularandmolecularlayersofthecerebellarcortexincreasedmyelinationinthemolecularlayerMitoticactivityandnecrosisareuncommonDysplasticCerebellarGangliocytoma正常小腦板層結(jié)構(gòu)破壞大量增生肥大的神經(jīng)節(jié)細(xì)胞侵蝕小腦皮層的顆粒層和分子層分子層髓
5、鞘化增加病理組織切片上有絲分裂活性及壞死少見PathologicFindingsDysplasticCerebellarGangliocytoma×200×100hypertrophicganglioncellsexpandingthegranularandmolecularlayersofthecerebellarcortexPathologicFindingsDysplasticCerebellarGangliocytoma免疫組化染色顯示神經(jīng)元特異性蛋白酶及突觸素陽性表達(dá)ImagingFindings-CTNCCTusuallyhypoattenuatedmaybeisoattenu
6、atedCalcificationisuncommonThinningoftheskullDysplasticCerebellarGangliocytomaCT平掃常為低密度但也可為等密度無特異性診斷困難鈣化少見偶可見顱板變薄ImagingFindings-MRIMRI：bestimagingmodalitycharacteristicappearancewithouthistopathologicalconfirmationtypicalappearances1.unilateralcerebellarmass2.non-enhancing3.middle-agedpatient4.ti
7、ger-stripedpatternDysplasticCerebellarGangliocytomaMRI是診斷的最佳方式特征性表現(xiàn)幾乎可在不需要病理證實(shí)的條件下做出明確診斷四條特征1.單側(cè)半球腫塊2.不強(qiáng)化3.成年患者4.虎斑征ImagingFindings-MRIcharacteristic:bandshyperintensityandisointensityonT2isointenseandhypointense
