谢谢各位的建议、意见及指正
A20-year-oldpresentswithprogressivebilateralsensorineuralhearingloss,pulsatiletinnitus,andrightfacialnervepalsy.
20岁，表现为进行性双侧感音神经性耳聋、搏动性耳鸣和右侧面神经麻痹。
CTscanoftemporalbonesshowsirregular,destructiveprocessesatbilateraltemporalbones.MRIofposteriorfossaisperformed.
颞骨的CT扫描显示两侧颞骨不规则形、破坏性突起。行后颅窝MRI检查。
(A)Anirregulardestructiveinfiltratingrighttemporalbonemass(whitearrow)arisingposteriortothecochleaintheretrolabyrinthinearea.(B)Anirregulardestructiveinfiltratinglefttemporalbonemassarisingfromtheretrolabyrinthinearea(whitearrow)extendingintothepetrousapex(whitearrowhead)andintothemiddleearcavity(blackarrow).(C)T1-weightedimageexhibitslarge,irregular,lobulated,bilateraltemporalbonemasses.ThesmallerrightsidedmassexhibitslowT1signal(whitearrow),whereasthelargerleft-sidedmasshaslobulationswithhighT1signal(blackarrow)aswellaslowT1signal.(D)T2-weightedimageshowsbilateralheterogeneousmasseswithhighT2-signalareassuggestiveofvascularmasses(whitearrows).SomeofthehighT1signallobulationsexhibitlowT2signal,suggestiveofhemorrhagicproducts(blackarrow).(E)PostcontrastimagesexhibitintenseenhancementoflowT1andhighT2signalareas(whitearrows).Verylittleenhancementisseenathemorrhagiclobulations.(F)Endovascularangiographyofrightexternalcarotidartery(ECA)showstumorblushsuppliedbyascendingpharyngealartery(blackarrow).
(A)位于右侧颞骨迷路后区耳蜗后方的不规则破坏性、浸润性肿块(白色箭头)。(B)左侧颞骨不规则的破坏性浸润性肿块，起源于迷路后区域(白色箭头)，延伸至岩尖(白色箭头)和中耳腔(黑色箭头)。(C)T1加权像显示双侧颞骨大的、不规则的、分叶状的肿块。较小的右侧肿块呈低T1信号(白色箭头)，而较大的左侧肿块呈分叶状高T1(黑色箭头)和低T1混杂信号。(D)T2加权图像显示双侧不均匀信号肿块，高T2信号区提示血管性肿块(白色箭头)。其中高T1、低T2分叶状病灶提示出血(黑色箭头)。(E)增强后图像呈低T1和高T2信号区明显增强(白色箭头)。分叶状出血区几乎没有强化。(F)右侧颈外动脉(ECA)血管内造影显示肿瘤由咽升动脉供血(黑色箭头)
DifferentialDiagnosis
?Endolymphaticsactumor(ELST):Destructivesofttissuelesionoriginatingintheretrolabyrinthineposteriorsurfaceofpetroustemporalbone,involvingsemicircularcanalsandvestibuleandspreadingtomiddleearcavity.
?Schwannomaofjugularforamen:Thelesioniscenteredinjugularforamenanddoesnotinvolvetheretrolabyrinthinetemporalbone.
?Cholesterolgranulomapetrousapex:ThelesioniscenteredinpetrousapexandthewholelesionexhibitshighT1signalonMRI.
?Metastaticdepositsfromrenalcellcarcinomaorpapillarythyroidcarcinoma:Theselesionsareverydestructiveanddonotexhibitanycalcificationoftheposteriorlimb.ItalsolacksthehighT1signalfocionMRI.
?Jugulotympanicumandglomusjugulareparaganglioma:Thelesioniscenteredinthejugularforamenandtheninvolvesthemiddleearanddoesnotinvolvetheretrolabyrinthinetemporalbone.
鉴别诊断·
?内淋巴囊肿瘤(ELST)：起源于岩颞骨迷路后表面的破坏性软组织病变，累及半规管和前庭，扩散至中耳腔。
?颈静脉孔神经鞘瘤：病变集中在颈静脉孔，不累及迷路后颞骨。
?岩尖胆固醇肉芽肿：病灶中心位于岩尖，MRI上整个病灶T1呈高信号。
?肾细胞癌或乳头状甲状腺癌的转移性沉积：这些病变具有很强的破坏性，后缘没有任何钙化。MRI上也缺乏高T1信号灶。
?颈静脉鼓室和颈静脉球副神经节瘤：病变集中在颈静脉孔，然后累及中耳，不累及迷路后颞骨。
EssentialFacts
?Slow-growingbutaggressiveadenomatousorcystadenomatoustumorarisingfromlowcolumnarorcuboidalcellularliningoftheendolymphaticsac.
?Mostlesionsarespermatic,but15%ofpatientswithvonHippel-Lindau(VHL)diseasedevelopELST.
?Sensorineuralhearinglossispresentinallthepatients.Othersymptomsincludefacialnervepalsy,pulsatiletinnitus,andvertigo.
?Treatmentincludes
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