骨其他腫瘤及瘤樣病變

骨腫瘤who分類山東大學(xué)醫(yī)學(xué)院病理教研室相磊骨其它腫瘤和瘤樣病變

①纖維結(jié)構(gòu)不良定義：–纖維結(jié)構(gòu)不良(FibrousDysplasia)也稱骨纖維異常增殖癥，是一種常見的骨發(fā)育異常，為發(fā)生在骨髓腔內(nèi)的良性纖維性骨病，可為單骨性或多骨性。因有染色體克隆性異常和基因活性突變，提示可能是腫瘤性病變。臨床特點(diǎn)：–大多數(shù)為單骨性（85%）–若多骨性伴有皮膚色素斑、內(nèi)分泌亢進(jìn)時(shí)，稱為Albright綜合癥–伴發(fā)肌內(nèi)粘液瘤時(shí)，稱為Mazabraud綜合癥–單骨性發(fā)病年齡范圍大，高峰年齡10~29歲；多骨性發(fā)病年齡小，常在10歲以前發(fā)病–可長期缺乏癥狀–生長發(fā)育停止后，生長逐漸靜止①纖維結(jié)構(gòu)不良部位：–單骨性最好發(fā)生于顱面骨，其次為股骨和脛骨以及肋骨–多骨性大多數(shù)發(fā)生在股骨、盆骨和脛骨①纖維結(jié)構(gòu)不良①纖維結(jié)構(gòu)不良①纖維結(jié)構(gòu)不良①纖維結(jié)構(gòu)不良①纖維結(jié)構(gòu)不良Pathologicfractureisnotuncommoninthislocation.Thereisfusiformswellingoftheproximalfemurwithahazyground-glassappearance①纖維結(jié)構(gòu)不良Roentgenographicappearanceoffibrousdysplasiainvolvingthefemoralshaft.Thelesionisextensive,radiolucent,andshowsscallopingofthecortex(arrow).Theappearancemaysimulatethatseeninchondrosarcoma.①纖維結(jié)構(gòu)不良①纖維結(jié)構(gòu)不良①纖維結(jié)構(gòu)不良①纖維結(jié)構(gòu)不良①纖維結(jié)構(gòu)不良Grossspecimenoffibrousdysplasiawithpronouncedcartilagedifferentiation(arrow)involvingtheproximalfemur.Thereareonlysmallfocioffibroustissuebetweentheislandsofcartilage.①纖維結(jié)構(gòu)不良①纖維結(jié)構(gòu)不良Classicappearanceoffibrousdysplasiaatlowpower.Thereisspindle-cellproliferationandboneformation.Thespindlecellsseemtoinvadethebonytrabeculaeandtherebygiverisetounusualformationsofbone(arrow).①纖維結(jié)構(gòu)不良Medium-powerappearanceoffibrousdysplasia,showingaspindle-cellproliferationanddeformedbonytrabeculaethatseemtoarisedirectlyfromspindlecells(arrow).①纖維結(jié)構(gòu)不良Fibrousdysplasiawithtypicalmetaplasticboneformation(arrow).①纖維結(jié)構(gòu)不良①纖維結(jié)構(gòu)不良①纖維結(jié)構(gòu)不良①纖維結(jié)構(gòu)不良①纖維結(jié)構(gòu)不良治療和預(yù)后：–部分骨骼彎曲或畸形需要手術(shù)矯正，刮除后需要植骨–一般不考慮放療，避免誘發(fā)肉瘤變–預(yù)后良好①纖維結(jié)構(gòu)不良②骨纖維結(jié)構(gòu)不良定義：–一種良性自限性纖維性骨病，幾乎只發(fā)生于脛腓骨，偶見尺撓骨。–組織形態(tài)類似纖維結(jié)構(gòu)不良，又稱皮質(zhì)內(nèi)纖維結(jié)構(gòu)不良–或組織形態(tài)與頜骨骨化性纖維瘤相同，又認(rèn)為是長骨的骨化性纖維瘤臨床特點(diǎn)：–脛骨近端中1/3最常見–10歲以前的兒童，15歲以后少見–腫脹和疼痛②骨纖維結(jié)構(gòu)不良影像學(xué)特點(diǎn)–骨皮質(zhì)內(nèi)偏心性的溶骨性改變，有硬化邊緣，骨膜反應(yīng)和軟組織受累少見。②骨纖維結(jié)構(gòu)不良②骨纖維結(jié)構(gòu)不良Roentgenogramofapatientwithosteofibrousdysplasia,showinginvolvementofthetibialcortex.Thelesionislong,andshowsmultiplelucentareasmixedwithareasofsclerosis(arrow).②骨纖維結(jié)構(gòu)不良Anothergrossspecimenofosteofibrousdysplasia,showinginvolvementofacortexsegmentandnoinvolvementofthemedullarycavity②骨纖維結(jié)構(gòu)不良治療和預(yù)后：–一般不主張手術(shù)，只有當(dāng)發(fā)生病理性骨折及嚴(yán)重彎曲畸形的情況下才需要手術(shù)②骨纖維結(jié)構(gòu)不良定義：–一種病因不明，發(fā)生在骨皮質(zhì)內(nèi)的非腫瘤性纖維組織和組織細(xì)胞增生性病變，系局部骨化障礙、纖維組織增生或纖維組織侵入皮質(zhì)所致，有人認(rèn)為是兒童發(fā)育中正常變異。–如果病變大，累及髓腔則稱為非骨化性纖維瘤–病變組織學(xué)形態(tài)十分類似于良性纖維組織細(xì)胞瘤，但兩者是完全不同的疾病。–大多數(shù)病例的病變可以自行消退③干骺端纖維缺損和非骨化性纖維瘤臨床特點(diǎn)–大約30~40%的骨骼生長期兒童X線檢查可發(fā)現(xiàn)小的無癥狀的干骺端皮質(zhì)缺損–病變從靠近骺板的部位開始發(fā)生，隨著骨骼的生長逐漸向骨干方向發(fā)展–大多無明顯癥狀③干骺端纖維缺損和非骨化性纖維瘤③干骺端纖維缺損和非骨化性纖維瘤③干骺端纖維缺損和非骨化性纖維瘤③干骺端纖維缺損和非骨化性纖維瘤③干骺端纖維缺損和非骨化性纖維瘤治療和預(yù)后–小的病變無需治療–較大的病變，為防止病理性骨折需刮除并植骨，或局部切除–預(yù)后良好③干骺端纖維缺損和非骨化性纖維瘤定義–尤因肉瘤屬于PNET中最不分化的一端，為小圓細(xì)胞肉瘤，有不同程度的神經(jīng)外胚葉分化；④尤因肉瘤和原始神經(jīng)外胚葉瘤臨床特點(diǎn)：–少見，占原發(fā)骨惡性腫瘤的7%–高峰年齡為10~20歲，大于30歲者極少見–局部疼痛和腫脹–發(fā)熱、血沉快，白細(xì)胞升高等類似骨髓炎癥狀–任何骨都可發(fā)生，但最常見的是長骨骨干–X線為蟲蝕狀破壞，可有骨膜反應(yīng)。常有軟組織腫塊④尤因肉瘤和原始神經(jīng)外胚葉瘤④尤因肉瘤和原始神經(jīng)外胚葉瘤SkeletalandagechartforEwing'ssarcoma.AlmostallpatientswithEwing'ssarcomaarebelowtheageof20years.Anyportionoftheskeletonmaybeinvolved,butthereisatendencytoinvolvetheaxialskeleton.Noticethepreponderanceofpelvicbonesandribs.④尤因肉瘤和原始神經(jīng)外胚葉瘤RoentgenogramofaEwing'ssarcomainvolvingtheproximalfibularshaft.Thelesionispurelylyticandhascompletelydestroyedalargesegmentofthebone,withproductionofalargesoft-tissuemass(arrow).④尤因肉瘤和原始神經(jīng)外胚葉瘤Ewing'ssarcomainvolvingthedistalfemoralshaft.Thelesionissubtleandformsasoft-tissuemasswithsaucerformationoftheunderlyingcortex(arrow).ThisappearanceispracticallydiagnosticofEwing'ssarcoma.RecurrentEwing'ssarcomaformingalargemassinvolvingthedistalfemur.Thelesionhasbrokenthroughthecortexandsurroundstheunderlyingbone.④尤因肉瘤和原始神經(jīng)外胚葉瘤④尤因肉瘤和原始神經(jīng)外胚葉瘤Low-powerphotomicrographofaEwing'ssarcoma.Thereareislandsofviabletumorcellssurroundedbypinkareasofnecrosis(arrow).FociofnecrosisarecommoninEwing'ssarcoma④尤因肉瘤和原始神經(jīng)外胚葉瘤High-powerphotomicrographofaEwing'ssarcoma,showinguniformroundtoovalnuclei.Thereisnotmuchvariationinthesizeandshapeofthenuclei.Thecytoplasmicboundariesareindistinct一種局部侵襲性生長傾向，或者說有低度惡性潛能的腫瘤，由成片腫瘤性卵圓形單核細(xì)胞和均勻分布的破骨細(xì)胞樣巨細(xì)胞構(gòu)成?占原發(fā)性骨腫瘤4~5%和原發(fā)性良性骨腫瘤20%?高峰發(fā)病年齡20~45歲?長骨骨端是典型發(fā)病部位，5%發(fā)生于扁骨，也可發(fā)生于骶骨。頜骨和手足骨的巨細(xì)胞瘤十分罕見?疼痛和腫脹為最常見癥狀。⑤巨細(xì)胞瘤⑤巨細(xì)胞瘤Mostpatientsareabovetheageof20years.Theendsoflongbonesareusuallyinvolved.⑤巨細(xì)胞瘤Thelesionispurelylytic,ispoorlymarginated(arrow),andextendstotheendofthebone.Thedistalfemuristhemostcommonlocationofthisdisease.⑤巨細(xì)胞瘤Thelesionextendstotheendofthebone(arrow)andisextremelydestructive.Thecortexisdestroyed,andthereisextensionintosofttissue.⑤巨細(xì)胞瘤Benigngiantcelltumorinvolvingthedistalfemur.Noticethetypicaldark-browncolorextendingtotheendoftheboneandalargesoft-tissuemass.⑤巨細(xì)胞瘤showingbenigngiantcellsandmononuclearcells⑤巨細(xì)胞瘤Thenucleiofthegiantcellsaresimilartothenucleiofmononuclearcells.治療和預(yù)后–約有1~2%組織學(xué)典型的巨細(xì)胞瘤會(huì)發(fā)生良性轉(zhuǎn)移，大多轉(zhuǎn)移到肺，多于術(shù)后一年左右發(fā)生。可以外科切除轉(zhuǎn)移灶。–大塊切除是理想的治療方案。病灶刮除，有1/3~1/2的病例會(huì)在5年內(nèi)復(fù)發(fā)。–放療可能會(huì)引起肉瘤變，一般不施行。手術(shù)難以清除的或復(fù)發(fā)性巨細(xì)胞瘤可以使用放療。⑤巨細(xì)胞瘤典型的良性巨細(xì)胞瘤區(qū)域+肉瘤區(qū)域?以前在同一部位腫瘤中有過病理證實(shí)的良性巨細(xì)胞瘤，現(xiàn)在發(fā)現(xiàn)有肉瘤區(qū)域⑥惡性巨細(xì)胞瘤⑥惡性巨細(xì)胞瘤⑥惡性巨細(xì)胞瘤Thepatientreceivedradiationtherapyforabenigngiantcelltumor.Thetumorwasdiagnosedapproximately13yearsbeforethisroentgenogramwastaken.Thereisamalignant-appearingdestructivelesioninthedistalfemur(arrow).Thereareareasofsclerosisresultingfrompriorirradiation.⑥惡性巨細(xì)胞瘤Malignantspindle-celltumorwithastoriformgrowthpattern⑥惡性巨細(xì)胞瘤Giantcelltumor(horizontalarrow)andaspindlingmalignancy(verticalarrow).治療和預(yù)后–治療原則和預(yù)后類似于骨肉瘤⑥惡性巨細(xì)胞瘤⑦動(dòng)脈瘤性骨囊腫?良性非腫瘤性骨病?充滿血液的囊腔和結(jié)締組織間隔?間隔內(nèi)含纖維母細(xì)胞、破骨細(xì)胞樣巨細(xì)胞和反應(yīng)性編織骨?原發(fā)性和繼發(fā)性?20歲以前青少年最多見?臨床主要表現(xiàn)為疼痛?絕大多數(shù)位于長骨干骺端和脊柱骨?X線為偏心性、膨脹性、邊界清楚的溶骨性骨質(zhì)破壞，可以有Codman三角形成，易被疑為惡性⑦動(dòng)脈瘤性骨囊腫⑦動(dòng)脈瘤性骨囊腫Thelesionissituatedeccentrically,withinvolvementofthecortex,andhasablow-outappearanceinthesofttissues(arrow).⑦動(dòng)脈瘤性骨囊腫Aneurysmalbonecystinvolvingthedistalfemora-tissuecomponentthatappearstobewellcircumscribed(arrow).⑦動(dòng)脈瘤性骨囊腫Thelesionhasdestroyedthebonecompletelyandformsasoft-tissuemass.Noticeaperipheralshellofossification(arrow).⑦動(dòng)脈瘤性骨囊腫Grossappearanceofananeurysmalbonecyst,showingspacesthatcontainbloodclots