成人孤立性骨嗜酸性肉芽肿45例临床分析

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利洪艺,温丽丽,王博,李洪波,王永谦,沈靖南,黄纲,谢显彪Vol.39(6):-

摘要:探讨成人孤立性骨嗜酸性肉芽肿(EG)的临床表现、影像与病理特点、治疗及预后。回顾性分析从年9月至年6月共45例成人孤立性骨EG患者的临床、影像学病理表现及治疗预后情况。男35例,女10例,中位年龄29岁,临床受累骨骼包括颅面骨(9例)、脊柱(11例)、肋骨(1例)、骨盆(8例)、锁骨(3例)、肩胛骨(2例)、肱骨(3例)及股骨(8例)等,平均随访81月。影像学特征为溶骨性破坏病灶,脊柱病灶中均未出现扁平椎改变,免疫组化多为CD1a、Langerin(CD)及S阳性。其中3例予保守观察,23例予手术治疗,8例接受小剂量化疗,其余11例行手术及术后辅助小剂量化疗,其中40例痊愈,5例复发后均再接受化疗,无进展生存时间(PFS)为3~个月,中位PFS为67个月。孤立性骨EG临床发病率很低,尤其成人更为罕见,临床表现无明显特异性,影像学表现易误诊,需重视病理检查,因成人孤立性骨EG的生物学行为比儿童更具侵袭性,治疗需个体化的同时可采取比儿童EG更积极的策略。

关键词:                                                                                                                                嗜酸性肉芽肿;骨;成人;治疗;预后

Abstract:Toanalyzethefeaturesofclinical,radiology,pathology,treatmentandprognosisofsolitaryeosinophilicgranuloma(EG)bonelesionsinadults.Weretrospectivelyreviewedtherecordsof45adultpatientswithsolitaryEGbonelesionsatourinstitutions.Thefeaturesofclinical,radiology,pathology,treatmentandprognosiswereanalyzed.Therewere35malesand10females,themedianagewas29years,themeanfollow-uptimewas80.89month.ThedistributionoflesionssiteswereCraniofacialbone(9cases),spine(11cases),pelvis(8cases),clavicle(3cases),scapula(2cases),humerus(3cases),femur(8cases).Radiographicfindingswerecharacteristicallyosteolyticlesions,vertebraplanawasnotfoundin11spinalcases,immunohistochemistryshowedpositiveinCD1a,Langerin(CD)andS.Ofthe45adultcases,3casesreceivedobservation;surgerywasperformedin23cases,lowdosechemotherapyweregivenin8cases,andtherestof11caseswereadministeredsurgeryandadjuvantchemotherapy.Fortycaseswerehealed,while5casesdevelopedprogressorrelapseandreceivedchemotherapyagain,therangeofprogress-freesurvival(PFS)was3-months(median:67months).SolitaryEGbonelesionsarerare,particularlyinadults.Insidiousclinicalpresentationandaggressiveradiologyfeaturecouldeasilydevelopmisdiagnose,accordinglypathologyisvitalfordiagnosis.Individualizedmanagementandmoreactiveinterventionwerere







































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