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1、小肠骨外尤因肉瘤外周原始神经外胚层肿瘤临床病理观察【摘耍】目的探讨小肠骨外尤因肉瘤/外周原始神经外胚层肿瘤的临床病理学特点、诊断及鉴别诊断。方法对1例小肠E-EWS/pPNET患者的临床特征及组织形态学和免疫组织化学染色进行分析，并复习相关文献。结果男性患者，61岁。临床表现为十二指肠腔内占位，累及胆总管下端及部分胰腺。镜下观察肿瘤组织由形态一致的小圆或卵圆形细胞所组成，胞质较少，细胞核呈圆形或类圆形、染色质浓染，病理性核分裂像易见，肿瘤细胞呈弥漫性实性片状密集分布，局部可见Homer-Wright菊形团样结构，伴有大片坏死。免疫组化
2、显示肿瘤细胞波形蛋白、CD99蛋白、神经元特异性烯醇化酶、突触素及CD56表达阳性，角蛋白癌胚抗原、白细胞共同抗原、结蛋白、高碘酸-雪夫（PAS）染色阴性。结论小肠E-EWS/pPNET是一种非常少见的小圆细胞高度恶性肿瘤，诊断主要依据病理形态学特征及免疫组化标记，筹神经内分泌标记物阳性并结合细胞遗传学特点对鉴别诊断有重要意义。【关键词】肿瘤；小肠；尤因肉瘤/外周原始神经外胚层肿瘤；免疫组化[Abstract]ObjectiveToinvestigateclinicopathologicalcharacteristicsanddiff
3、erentialdiagnosisofEwing,sSarcoma/primitiveneuroectodermaltumor（EWS/卩NET）insmallintestine.MethodsOnecaseofEWS/PNETinsmallintestinewasstudiedbyclinicopathologicfeatures,histochemistry,andimmunohistochemistry,withreviewoftherelevantliteratures.ResuItsThepatientwasa61year-
4、oldman.Theclinicalsymptomwasduodenalmasseswithinvadingcommonbileductandpartialpancreas・Histologically,thetumorcellswerediffusiveandunifonnpatchyintensive,andthetumorcellscontainedonlyafewcytoplasm・Manyundifferentiatedroundcellswithroundorirregularhyperchromaticnuclei.Ab
5、undantmitoticfigureswerepresent.ThecelIsdistributeddiffusivelyorformedlobulatedstructures(Ilomer-Wrightrosette)withextensivenecrosis.ImmunohistochemicalanalysisshowedpositiveexpressionforVimentin,CD99,Neuronspecificenolase(NSE),SynandCD56proteinintumorcells.Thetumorcel1
6、spresentednegativeforcytokeratin(CK),CK20,CK8/18,CEA,LCA,Desmin,CgA,CD117,CD34,S-100protein,IIMB45,a-inbin,insulin,gastrin,calcitoninandglucagon.TheKi67labelingindexwas90%・PASspecificstainwasnegative・ConclusionEWS/PNETinsmallintestineisarareandhighlymalignantsmal1roundc
7、elltumor,andthediagnosismustbasedonpathologicfindingsandimmunohistochemicalphenotypes・PositiveexpressionforCD99,CD56andNSEcombinewithmoleculargeneticstudymaybevaluableindifferentialdiagnosis.【Keywords]Neoplasma；Smal1intestine；Ewing'ssarcoma/primitiveneuroectodermaltumor
8、；Iminunohistochemistry原始神经外胚层肿瘤是一类罕见的小圆细胞恶性肿瘤，根据发病部位不同可分为外周性PNET和中枢性PNETo近年研究表明，外周性卩NET与骨组织的尤因肉瘤有许多相似之处，如两者都具有
