骨腫瘤

Chapter1SummaryConception：凡發生在骨內或起源於骨各種組織成分的腫瘤，不論原發性，還是繼發性或轉移瘤，統稱為骨腫瘤。Theincidenceofprimarymalignantboneneoplasm(excludingmyeloma)isabout1caseper100,000individualsperyear.Truebenignbonetumorsatabouthalfthisfrequency.Classification

目前的分類皆基於細胞來源.

我國自已的骨腫瘤分類特點是:1.將骨腫瘤分為良性、中間性和惡性三類.2.將骨巨細胞歸納於組織細胞來源內.3.採用臨床、病理和X線三結合.HistologicalclassificationofbonetumorsDifferentiationorhistogenesisbenignLow-grademalignanthigh-grademalignantFibrousandhistiocyticGiantcelltumorHistiocyticfibromaGrade1and2fibrosacomaGrade3and4fibrosacomaCartilaginousChondromaChondroblastomaPeripheralCSPeriostealCSDefferentiatedCSMesenchymalCSOsseousOsteoblastomaLow-gradecentralOSSmallcellOSNervousNeurofibromaPeripheralneuroectodermictumor神經外胚層CS=chondrosarcoma,OS=OsteosacomaTumor-likeSpace-Occupyinglesion(瘤樣病損)

1.Simplecyst(孤立性骨囊腫)2.Aneurysmalbonecyst(動脈瘤性骨囊腫)3.Eosinnophilicgranuloma嗜酸性肉芽腫

4.Fibrousdysplasiaofbone骨纖維異樣增殖癥.5.Synovialchondromatosis滑膜肉瘤

6.Pigmentedvillonodularsynovitis色素沉著絨毛結節性滑膜炎

Tumors:benignversusmalignantPatientwithbonetumorsusuallypresentwithpain;however,theymaypresentwithapainfulmassorevenwithapainlessmass.Thesymptomsandsignsdependontheindolenceoraggressivenessofatumoranditsinteractionwithagivenhost.Themostcharacteristicsymptomassociatedwithbonetumorsispainpresentatnight.Asthispainbecomesmoreprogressive,itisusuallysevereenoughtowakepatientsfromtheirsleep.

Incertaintumors,theonsetandreliefofpainischaracteristicenoughtomakeaspecificdiagnosis.Forexample,inosteoidosteoma,aspirinusuallyrelievesthenocturnal（夜間的）

painassociatedwiththetumor.Thisisbelievedtobereletedtotheuniqueinnervation(神經支配)ofthislesioncombinedwithitsbiosynthesisofprostaglandins.GrowthpatternsMalignantbonetumorspossesscharacteristicsgrowthpatternsthathelptodistinguishthemfromabenigntumor.Ingeneral,malignanttumorsgrowataratefasterthanthatatwiththesurroundinghostboneisreabsorbed.Inaddition,theyusuallydisplayinvasivelocalextension(擴張)intothesurroundingosseousandinterosseous（骨間的）

tissues.Thispatternofgrowthissometimesreferredtoaspermeative.GrowthpatternsDespitethesubtlechangesoftenseenonplainradiographs,thisgrowthpatternisclinicallyassociatedwiththemostmalignantbehavior.Veryoften,thereisassociatedtumorextensionintotheadjacentsofttissuestoproduceanextraosseoustumormassinthecompleteabsenceofdemonstriblecorticaldestruction.EffectoftumorsBonetumorsaffectindividualsofallages,butingeneral,primarybonetumorshavetheirgreatestfrequencyinyoungindividuals.Thenotableexceptionstothisrulearechondrosarcoma,whichismostfrequentlyseenintheyoungindividuals.Metastaticcarcinoma,althoughnotaprimarybonetumor,isveryimportantinconsiderationsofskeletaltumorsbecauseitisthemostfrequentlymalignanttumorstoaffectbones.EffectoftumorsThemajorityofindividualswhodieofwidespreadmetastaticcarcinomaintheirskeletoneventhoughtheymaynotpresentwithbonelesionsclinically.Metastaticcarcinomausuallyaffectsmorethanonesitesinasinglebone,anditoftenaffectsmorethanjustonebone.EffectoftumorsWhileanypotionofabonemaybeaffectedbyabonetumor,certainneoplasmsreproduciblyaffectparticularareasofabone.Forexample,osteosarcomamostfrequentlyarisesinthemetaphysesofrapidlygrowinglonebone.Itisnotsurprising,then,thatosteosarcomaisseenmostofteninthedistalfemurandproximaltibiaofteenagersandthatithasgreaterfrequentlyintallerindividualsthaninshorterones.EffectoftumorsBecausethemostcommonprimarybonetumorproduceextracellularosseousorcartiginousmatrix,secondarymineralizationorossificationmaybesuperimposedonbonedistruction.Themostfamillarofthesereactionsisthediscontinuoussingleperiostealnewbonelineopenatoneend,termedCodman`striangle.DiagnosisThehistory,physicalexamination,andplainradiographsarecriticalinmakingdecisionsaboutanybonelesion.Whenaspecificdiagnosiscannotbemade,additionalradiographictestsareusuallynecessarybeforetakingabiopsysample.Thebiopsyisthelasttesttobedoneinmostcircumstances.Imagingstudies-radiographs1.反應骨:有些腫瘤表現為骨的沉積.2.腫瘤骨:有些腫瘤細胞產生類骨.3.Codman三角:骨膜被腫瘤頂起,骨膜下產生新骨.4.“蔥皮”現象:骨膜的掀起是階段性的,這樣就形成板層狀骨沉積.

5.“日光射線”形態:惡性腫瘤生長迅速,超出骨密質範圍,同時血管隨之長入,從密質骨向外放射,腫瘤骨與反應骨乃沿放射血管方向沉積.6.病理性骨折:骨因破壞性吸收而容易骨折.7.硬骨性轉移:激發骨成骨性反應.ImagingstudiesComputedTomography

可提供橫斷面影像，因而可確定瘤骨及軟組織侵犯情況MagneticResonanceImaging

能更清楚反映軟組織情況，並可初步判定腫瘤的性質生化測定生化檢查:血沉、血磷、鹼性磷酸酶、酸性磷酸酶、總蛋白濃度。溶骨性腫瘤---血鈣升高；成骨性腫瘤---鹼性磷酸酶升高；前列腺癌晚期骨轉移---酸性磷酸酶升高；漿細胞骨髓瘤---尿本周氏蛋白（Bence-Jonesprotine）陽性Biopsy

Thebiopsyisthelasttesttobedoneinmostcircumstances.Openbiopsy切開活檢切取式切除式

Needlebiopsy穿刺活檢

Needlebiopsyisarelativelyinexpensiveprocedurewithalowriskofhemorrhageandinfection.外科分期用來評估骨腫瘤治療,已被公認合理,有效.外科分期是外科分級G,外科區域T和遠處轉移M三結合.G:G0良性;G1低度惡性;G2高度惡性.T:T0囊內;T1間室內;T2間室外.M:M0無轉移;M1為轉移.Treatmentprinciple1.良性骨腫瘤可採用保守治療2.良性骨腫瘤的手術治療刮除植骨術：塗抹藥物或燒灼創面殺滅殘留瘤細胞外生性骨腫瘤切除術：切除要徹底，防止復發3.惡性骨腫瘤：採取以手術為主的聯合治療方法,術前術後化療、放療、免疫療法、中藥等.手術應按外科分期來選擇手術界限和方法.Preservelimbandamputition

保肢手術與傳統截肢術的生存率和復發率相同。手術應首先考慮肢體的保留，儘量切除瘤體，而將截肢放在慎重的地位。同時應考慮將來假肢的安裝問題。Indicationsforlimbsalvagesurgery：1.Tumorresectablewithwidemargin(5cmbone,1.0cmsofttissue)，2.Skeletalreconstructionispossible，3.Soft-tissuereconstructionispossible，4.Resultingfunctionshouldbebetterthananamputation，5.resultmustbecosmeticallyandemotionallyacceptable.Limbsalvagesurgery:principles1.tumorresection2.skeletalreconstruction3.soft-tissuereconstructionContraindicationsforlimbsalvagesuegery:

1.saferesectionnotpossible，2.Arterialreconstructionnotpossible，3.Involvementofmajornerve(s)(e.g,sciatic)4.Late-stagedisease.5.Pathologicalfracture.Reconstructionafterlesionalexcision瘤骨骨殼滅活回植術異體骨半關節移植術人工假體置換術Chemotherapy可提高療效。治療有效的表現：疼痛減輕，腫物體積變小，關節活動改善，生化檢查恢復正常，影像學上瘤體變小，輪廓清楚，病灶鈣化或骨化，腫瘤性新生血管減少或消失。Radiotherapy可強有力的影響惡性腫瘤細胞的繁殖能力。病變廣泛不能手術者，可單獨放療。尤文肉瘤對放療敏感。骨肉瘤對放療不敏感。Chaptertwo

BENIGNBONETUMORSOsseous骨瘤、osteoidosteoma骨樣骨瘤、Cartilaginousosteoma骨軟骨瘤Chondroma軟骨瘤Cartilaginousosteoma

骨軟骨瘤較常見，結構為骨組織和其上的軟骨帽，主要由表面生長的軟骨帽逐漸骨化而成，因此腫瘤應屬軟骨源性。多發於青少年，常見於長骨的幹骺端?？砷L期無癥狀，壓迫周圍組織及表面滑囊發炎可出現疼痛等癥狀。

Ｘ線表現：在幹骺端可見骨性隆起，其皮質與松質骨與正常骨相連，可帶蒂或無蒂若有癥狀或生長過快，應切除，範圍包括基底四周正常組織及滑囊、軟骨帽，瘤本身ClinicalfeaturesandpresentationEnchondromaEnchondroma,themostfrequenttumorofboneofthehand,rarelyexhibitsaggressivebehaviorandissometimesreferredtoasacartilaginoushamartoma(錯構瘤)Symptoms:nonspecific,pathologicfracture,painisanominoussymptomsandsuggeststhatthetumorisgrowingandmaybemalignant.第三節

Giantcelltumur

骨巨細胞瘤

Diagnosis1.年齡:20-40歲的成年人。性別差別不大2.部位:多見長管骨的骨端,好發於股骨下端和脛骨上端3.按分化程度分為三級：Ⅰ級偏良性，Ⅱ級為侵襲性，Ⅲ級為惡性4.局部漸腫脹、疼痛、有乒乓球樣感。影響關節活動5.X線表現;骨端偏心、膨脹、肥皂泡樣影.

Treatment1.Ⅰ～Ⅱ級者：手術局部刮除加物理或化學處理，松質骨或骨水泥填塞.若復發，廣泛切除和大塊骨或假體植入.2.Ⅱ～Ⅲ級者：應廣泛或根治切除或截肢.化療無效.。放療雖有效，易肉瘤變，不可取.ChapterFour

MalignantBoneTumorsSummaryPrimarytumorsofbonearerare,andtheyarelesscommonthanmetastaticlesions,generallycarcinomas.Bonetumorsaredefinedbybothhistologyandsurgicalstaging.Osteosarcomaisamalignanttumorcomposedofmesenchymalcellsthatproduceosteoidandimmaturebone.Ewing′ssarcomaisamalignanttumorcomposedofpoorlydifferentiatedsmallroundcells.Chondrosarcomaisamalignanttumorcomposedofchondrocytesthatfillthemedullarycanalandinvadecancellousbone.Malignantfibroushistiocytomaisanintramedullary,highgradesarcoma,mostcommonlylocalizedinthelongbones.

osteosarcoma

骨肉瘤Diagnosis1.年齡:15—25歲年青人.2.部位:常見長管狀骨的幹骺端,高度惡性.3.局部疼痛,開始輕,間歇性,以後重,持續性.4.局部皮溫高,淺靜脈怒張,關節活動受限.5.X線表現:幹骺端破壞Codman三角,“日光射線”現象,腫瘤軟組織影.

Treatment1.術前大劑量化療，然後根治切除後置入假體保肢或截肢，術後繼續化療.2.骨肉瘤惡性度高,早診斷、早治療，才能提高5年存活率.

Ewing'ssarcoma

尤文肉瘤Diagnosis1.年齡:兒童.2.好發部位:股骨、脛骨、尺骨等.3.進行性疼痛,夜間重,伴發燒.4.局部腫塊,有壓痛.5.化驗:白細胞多,血沉快.X線蔥皮反應.Treatment

對放療極為敏感、預後差；截肢或保肢手術效果也不理想；化療也很有效，能延緩轉移?，F採用放療加化療和手術的綜合治療

脊索瘤Diagnosis1.好發部位:只發生於脊椎和顱底蝶枕部，骶尾椎多見2.生長緩慢,癥狀很輕，主要是疼痛和腫塊3.可出現壓迫神經和器官的癥狀壓迫骶神經、直腸和膀胱，產生相應癥狀4.X線:溶骨性破壞,膨脹性病變，無骨膜反應Treatment1.部位不同,治療不一樣，以手術為主2.不能手術切除或切除不徹底，可行放療3.復發率高，轉移晚，化療無效

simplebonecyst

骨囊腫unicameralbonecystTheunicameralbonecystisprobablynotatureneoplasm.Thiscystsoccurusuallyinthefirsttwodecadesof