骨纤维结构不良临床病理分析.pdfVIP

维普资讯 临床与实验病理学杂志 JClinExpPathol2008Jun；24(3) ·327 · 骨纤维结构不 良临床病理分析 谢小志 ，王宗敏 ，谢丽微 摘要：目的 探讨骨纤维结构不 良的临床病理特点 ，提高对该疾病的认识。方法 观察6例骨纤维结构不 良的临床特点及病 理形态学特征 ，并进行组化和免疫组化染色。结果 该肿瘤特征性地累及婴儿和儿童的胫骨中段前面的皮质，影像学上病变 境界清楚 ，骨皮质变薄、膨胀甚至消失 ，边缘有硬化带，组织学上骨纤维结构不 良有不规则的编织骨碎片，其边缘常衬覆着板 层骨，板层骨由轮廓分明的骨母细胞产生。免疫组化染色显示6例中3例有梭形细胞 CK散在阳性，未见CK阳性的上皮细胞 巢，S-100阴性。骨纤维结构不 良的自然病程是在 l0岁以前逐渐生长 ，到 l5岁左右逐渐 自行消退并康复。l0岁以下术后复 发率高。结论 骨纤维结构不 良有特征性的病理改变及临床表现，应注意与纤维结构不 良等病变鉴别。 关键词：骨肿瘤；骨纤维结构不 良；病理学，临床 中图分类号：R738．1 文献标识码：A 文章编号：1001—7399(2008)03—0327—03 Osteofibrousdysplasia：aclinicOpathOlOgicalstudy XIEXiao—zhi，WANGZong—min，XIE Li—wei (DepartmentofPathology，YuyingChildrenHospital，WenzhouMedicalCollege，Wenzhou 325027，China) Abstract：Purpose Tostudytheclinicalandpathologicalfeaturesofosteofibrousdysplasia(OFD)．Methods SixcasesofOFD wereanalyzedwithreview ofclinicopathologicaldata．Immunohistochemicalstainingwasperformedinthisstudy．Results OFD typi— cally involvedcorticalboneoftheanteriormid—shaftofthetibiaduringinfancyandchildhood．Itwasassociatedwithathining，expan— dingorevenmissingcortex．Theexpandingcortexwasoftenscleroticallyrimmednearthemedullarybone．Thehistopathologiefindings inOFD wereirregularrfagmentsofwovenboneoftenrimmedby lamellarlayersofbonelaiddownbywelldefinedosteoblasts．Immu— nostainingshowedthatspindlecellswerepositiveforCK in3of6cases，butnoendothelialcellnestswerefoundwhichwerepositive ofrCK．AllcaseswerenegativeofrS-100．Thenaturalhistory ofOFDwasthatofgradualgrowthduringthefirstdecadeoflifewithsta— blizationatabout15yearsofageofllowedbyhealingorspontaneousresolution，surgery undertheageof10hadahighrecurrencerate． Conclusions OFD hascharacteristichistopathologicimageandclinicalprogress，whichshouldbedistinguishedrfom thediseaseoffi— brousdysplasia． Keywords：boneneoplasms；osteofibrousdysplasia；pathology，clinical 骨纤维结构不 良(osteofibrousdysplasia，OFD) 病理主任医师阅片