#论著#作者单位:200032上海,复旦大学附属儿科医院病理科(陈莲);复旦大学附属华山医院神经病理科(汪寅);复旦大学附属肿瘤医院病理科(朱雄增)通信作者:汪寅(Emai:lyinwang88@hotmai.lcom)毛细胞黏液样型星形细胞瘤的临床病理学观察陈莲汪寅朱雄增【摘要】目的探讨毛细胞黏液样型星形细胞瘤的临床病理学特点。方法观察3例毛细胞黏液样型星形细胞瘤的光镜形态,采用EnVision法免疫组织化学标记胶质纤维酸性蛋白(GFAP)、CD34、K-i67,并结合文献复习分析其生物学行为。结果3例均为女性,年龄分别为9个月、10岁和19岁。1例发生于视交叉,2例发生于第三脑室(近鞍区部位)。镜下由双极性的梭形细胞组成,间质内含有大量的黏液。肿瘤内的血管明显增生,部分区域内瘤细胞显示以血管为中心性生长。3例切片内均未见到经典型毛细胞星形细胞瘤中的双相性形态,也未见Rosenthal纤维和嗜酸性颗粒小体。免疫标记显示,瘤细胞强阳性表达GFAP,瘤细胞增殖指数(K-i67)<1%。CD34标记显示清晰瘤内增生的血管。结论毛细胞黏液样型星形细胞瘤是毛细胞星形细胞瘤的一种独特亚型,在组织学上与毛细胞星形细胞瘤有不同之处,临床上较毛细胞星形细胞瘤更具有侵袭性。部分病例也可发生于儿童和青少年。GFAP标记有助于该瘤的诊断和鉴别诊断。【关键词】星形细胞瘤;脑肿瘤;诊断;预后Pilomyxoidastrocytoma:aclinicopathologicstudyofthreecasesCHENLian*,WANGYin,ZHUXiong-zeng.*DepartmentofPathology,ChildrencsHospital,MedicalSchool,FudanUniversity,Shanghai200032,ChinaCorrespondingauthor:WANGYin(DepartmentofNeuropathology,HuashanHospital,FudanUniversity,Shanghai200032,China,Email:yinwang88@hotmail.com)【Abstract】ObjectiveTostudytheclinicopathologicfeaturesofpilomyxoidastrocytoma(PmA).MethodsTheclinicalandpathologicfeaturesin3casesofPmAwereanalyzed.Immunohistochemicalstudyforglialfibrillaryacidicprotein(GFAP),CD34andK-i67wasperformedonparaffin-embeddedsectionsbystandardEnVisionmethod.ResultsAllthe3casesoccurredinfemalepatientsattheageof10months,10yearsand19yearsrespectively.Twocaseswerelocatedinthethirdventricle,whiletheremainingcasewaslocatedattheopticpathway.Histologically,thetumorwascomposedofbipolarspindlecellssettinginastrikinglymucinousbackground.Therewasamarkedproliferationofvesselswithinthetumor.Insomeareas,thetumorcellsexhibitedanangiocentricgrowthpattern.ThebiphasicpatternnotedinaclassicpilocyticastrocytomawasnotfoundinPmA.Rosenthalfibersandeosinophilicgranularbodieswerealsonotidentified.ImmunohistochemcalstudyshowedthatthetumorcellswerediffuselypositiveforGFAP.TheK-i67labelingindexmeasuredlessthan1%.CD34highlightedmainlythevascularnetworks.ConclusionsPmAisadistinctivevariantofpilocyticastrocytomawithsubtlehistologicdifferences.Comparedwithconventionalpilocyticastrocytoma,PmAbehavesmoreaggressively.Somecasestendtooccurinolderchildrenandadolescents.ImmunohistochemicalstudyforGFAPishelpfulindifferentialdiagnosis.【Keywords】Astrocytoma;Brainneoplasms;Diagnosis;Prognosis毛细胞黏液样型星形细胞瘤(pilomyxoidastrocytoma)是毛细胞星形细胞瘤(pilocyticastrocytoma)的一种特殊亚型,主要发生于2~3岁幼儿的鞍上区[1]。毛细胞黏液样型星形细胞瘤与毛细胞星形细胞瘤的关系尚不明确,尽管两者在发病年龄上相似,但在组织学上和生物学上却有着不同之处。我们对3例毛细胞黏液样型星形细胞瘤进行回顾性研究,探讨其临床病理学特点和鉴别诊断,并结合文献复习,分析其生物学行为。#727#中华病理学杂志2006年12月第35卷第12期ChinJPatho,lDecember2006,Vol35,No.12图1例1,毛细胞黏液样型星形细胞瘤,矢状位T1WI示肿块呈低信号(箭头所示)图2例1,冠状位T2WI示肿块呈高信号(箭头所示)图3例2,轴位第三脑室肿块T1WI呈不均匀略高信号(箭头所示)图4例2,矢状位T1WI增强示肿块明显强化(箭头所示)图5例3,...
