骨肉瘤(Osteosarcoma).ppt

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1、The 3rd Affiliated Hospital of Kunming Medical College1骨肉瘤（Osteo sarcoma）许建波昆明医学院第三附属医院云南省肿瘤医院The 3rd Affiliated Hospital of Kunming Medical College2骨肉瘤起源于未分化的骨纤维组织在发展过程中可以形成骨组织及其骨样组织又称为成骨肉瘤The 3rd Affiliated Hospital of Kunming Medical College3骨肉瘤统计数据占原发性骨肿瘤16.79，占原发性恶性骨肿瘤首位（40.51）The 3rd Affiliate

2、d Hospital of Kunming Medical College4骨肉瘤的分类1939年，美国学院外科学会将本病分为髓腔型、骨膜型、成骨型、溶骨型及毛细血管扩张型种根据肿瘤的骨内位置分为骨内型、中心型、骨膜型、骨皮质旁型、骨表面型等根据肿瘤成分分为骨母细胞型、软骨母细胞型、纤维母细胞型、纤维组织细胞型、毛细血管扩张型、软骨细胞型及小细胞型等根据肿瘤细胞分化程度分为高度恶性、中度恶性、低度恶性三种类型根据病灶分布分为单发型及多发型根据病程分为原发型及继发型。The 3rd Affiliated Hospital of Kunming Medical College5典型骨肉瘤是最常发生

3、在骨的原发性恶性肿瘤起源于骨内，由产生骨质的间质细胞生成肿瘤骨较多偶见肿瘤骨稀疏及散发The 3rd Affiliated Hospital of Kunming Medical College6临床表现年龄与性别好发部位症状与体征The 3rd Affiliated Hospital of Kunming Medical College7The 3rd Affiliated Hospital of Kunming Medical College8年龄与性别好发于男性，男与女之比为1.5：175病例在1030岁发病The 3rd Affiliated Hospital of Kunming M

4、edical College9好发部位在四肢长骨的好发部位为干垢端和骨干旁最常见为股骨远端和胫骨近端其次为股骨近端，约3/4的骨肉瘤出现在膝或肩再其次为股骨近端，股骨干和骨盆前臂和跗骨不常见生长软骨的长期存在可推迟肿瘤入侵骨髓The 3rd Affiliated Hospital of Kunming Medical College10症状与体征初期常无典型的症状在数周内，病情进展展，疼痛加剧局部皮肤温度增高邻近关节功能障碍，红肿、浅表静脉网状怒张骨质溶解，并发病理骨折。局部淋巴结并不增大和增多体重下降和贫血现象，预示肿瘤开始/已经转移The 3rd Affiliated Hospital o

5、f Kunming Medical College11线检查对骨肉瘤性质判断最准确的影像学方法。肿瘤扩散破坏骨质骨膜隆起穿破骨膜新生骨/骨样组织关联因素：年龄、肿瘤部位、生长速度、发展方式及新生骨情况The 3rd Affiliated Hospital of Kunming Medical College12病变早期骨内局部性的改变，溶骨改变/混合性改变/硬化（成骨）影像。骨膜反应，一般均呈薄层或分层改变，或伴有小的放射样骨针。The 3rd Affiliated Hospital of Kunming Medical College13病变发展骨皮质被破坏并多处穿破软组织内肿瘤性肿块阳光射

6、线影像高度不透过放射线的结节和分支状影像。Codman三角洋葱皮样改变The 3rd Affiliated Hospital of Kunming Medical College14CT检查了解肿瘤在软组织中扩展的情况与大血管或重要脏器的关系对关节组织和关节腔的侵犯诊断手术预后The 3rd Affiliated Hospital of Kunming Medical College15MRI检查检测肿瘤在松质骨和循骨髓腔扩展情况显示遗漏的肿瘤转移了解肿瘤与其周围软组织关系The 3rd Affiliated Hospital of Kunming Medical College16血管造影术

7、显示由于肿瘤性栓塞所致的静脉梗阻肿瘤血供和滋养血管分布情况常可见到“血池”现象The 3rd Affiliated Hospital of Kunming Medical College17骨扫描明确显示骨肉瘤的境界及其扩展情况发现遗漏的转移或在其他部位的病灶The 3rd Affiliated Hospital of Kunming Medical College18肺转移病灶呈圆形，多处分布高密度影，大小不一，密度一致一般并不钙化，偶尔表现为散发的钙化显示在X线放射影像呈阴性的转移灶The 3rd Affiliated Hospital of Kunming Medical College

8、19病理学检查大体病理学特征组织病理学特征The 3rd Affiliated Hospital of Kunming Medical College20大体病理学特征在硬化区则以象牙质样硬固为特征。起源于肿瘤骨的小梁骨结构呈现带状、束状和厚密的网状。肿瘤组织具有侵袭性当生长软骨仍然存在时，往往不致被肿瘤穿破The 3rd Affiliated Hospital of Kunming Medical College21组织病理学特征肿瘤由产生类骨质和骨质的肉瘤组织细胞组成肿瘤细胞多种 多样可见骨样组织（类骨质）或骨质（组织）沉积新生骨的结构紊乱，肿瘤骨在宿主骨的髓腔内扩散，宿主骨的小梁骨或被存

9、留的组织均包藏在变异的骨组织中，此为骨肉瘤的诊断标志。The 3rd Affiliated Hospital of Kunming Medical College22实验室检查早期出现贫血及白细胞升高碱性磷酸酶增高切除肿瘤后，碱性磷酸酶可立即降低复发或转移，碱性磷酸酶再次增高The 3rd Affiliated Hospital of Kunming Medical College23诊断严格遵循 临床、影像学及病理学三结合的原则The 3rd Affiliated Hospital of Kunming Medical College24鉴别诊断一般均可经套管穿刺活进、检而确诊。并非所有的骨

10、肉瘤的放射线影像均典型组织学表现特殊，个别情况下，病理也难以确诊The 3rd Affiliated Hospital of Kunming Medical College25分期分期的确定对制定以后的治疗方案有极其重要的意义具体分期依赖于临床、影像学及病理学检查确定90以上的典型骨肉瘤在就诊时即已侵蚀骨皮质并侵袭软组织，属于间室外的期类型部分病人就诊时已存在肺部转移，属于期少部分病人就诊时病损局限于间室内，属于 1期The 3rd Affiliated Hospital of Kunming Medical College26治疗是以手术为主的综合治疗手术治疗化学治疗放射治疗免疫及其他辅助治

11、疗The 3rd Affiliated Hospital of Kunming Medical College27手术治疗保肢术截肢术：高位截肢或关节离断术The 3rd Affiliated Hospital of Kunming Medical College28截肢术仍是治疗骨肉瘤的重要手段应用于期以及期不伴肺外转移的骨肉瘤一经确诊，应尽快实施。优点最大限度地切除原发病灶手术操作简单术后即可尽快施行其他辅助治疗期患者，如化疗反应良好，应从严掌握The 3rd Affiliated Hospital of Kunming Medical College29保肢术手术指征骨骼发育已基本趋于成

12、熟，年龄y Enneking外科分期期术区组织条件要求：无主要的血管神经受累、病理性骨折、局部感染和弥漫性皮肤浸润。能行边缘切除肿瘤保肢重建后，预期功能好于假肢保肢术后复发率与生存率估计不高于截肢患者及其家属均有保存肢体的强烈愿望。The 3rd Affiliated Hospital of Kunming Medical College30保肢术手术类型瘤段切除术人工假体置换同种异体骨移植复合人工关节移植肿瘤骨灭活再植自体骨移植12岁以下的患儿瘤段切除关节固定术化疗骨延长术、Salz（沙氏）旋转截骨重建术The 3rd Affiliated Hospital of Kunming Medic

13、al College31肺转移的手术治疗根治切除原发病灶肺转移病灶可考虑行手术切除转移病例的化疗问题初次术后1y，沿用以前的化疗方案/药物The 3rd Affiliated Hospital of Kunming Medical College32比较手术结合辅助化疗单纯的手术治疗，骨肉瘤的治愈率已有明显提高。经影像学检查排除远处转移，手术截肢化疗约有80的患者在术后612个月发生肺部转移，这说明骨肉瘤患者在就诊时，微细转移到肺的发生率相当高The 3rd Affiliated Hospital of Kunming Medical College33单剂化疗大剂量T7.5+CF解救疗法可使

14、：3040骨肉瘤患者的肿瘤缩小,年以上无瘤生存率约为40单剂ADM可使：2030患者肿瘤缩小,年无瘤生存率也约为40The 3rd Affiliated Hospital of Kunming Medical College34联合化疗多药联合化疗较单剂药物化疗更为有效大剂量 MTCF解救合并阿霉素的联合化疗方案5年生存率达59顺铂、博来霉素、环磷酸胺和更生霉素也是常用药物，可提高化疗效果多药联合配伍方案相继出台The 3rd Affiliated Hospital of Kunming Medical College35传统化疗术后静脉途径给药The 3rd Affiliated Hospi

15、tal of Kunming Medical College36新辅助化疗概念：1982年，由Rosen提出 1990年以来，已成为治疗骨肉瘤的标准方法术前化疗，原发病灶手术切除后经静脉化疗观察瘤灶细胞坏死率，优化术前化疗方案The 3rd Affiliated Hospital of Kunming Medical College37临床随访发现肿瘤细胞坏死率达到90，术后可沿用术前化疗方案肿瘤细胞坏死率低于90，术后化疗方案需予以调整肺转移经联合治疗，长期无瘤生存率为2240复发/转移者再手术，辅助化疗仍可获得2040的缓解生存率原发灶转移灶化疗反应存在差异The 3rd Affiliat

16、ed Hospital of Kunming Medical College38放疗放疗远没有化疗研究深入骨肉瘤放疗目前仍争议颇多The 3rd Affiliated Hospital of Kunming Medical College39骨肉瘤的预后病程短，进展快，一经确诊，自然病程6070），延滞转移The 3rd Affiliated Hospital of Kunming Medical College40毛细血管扩张性骨肉瘤（telangiectatic.osarcoma）又称出血性骨肉瘤属高度恶性骨肿瘤发病约占骨肉瘤的The 3rd Affiliated Hospital of

17、Kunming Medical College41临床表现 其发病与典型骨肉瘤在性别和 年龄方面并无差别病灶常起源于骨干，侵犯干垢端以股骨远端、胫骨、肱骨、腓骨、肩肿骨及骨盆常见本病进展迅速病理骨折较典型骨肉瘤多The 3rd Affiliated Hospital of Kunming Medical College42影像学检查 X-线表现为骨质溶解，以及显著的侵袭征象，骨膜成骨反应少见CT及MRI检查可显示肿瘤内部的液平面，提示病灶内有出血The 3rd Affiliated Hospital of Kunming Medical College43病理学检查大体病理学特征肿瘤由充满血液

18、和凝血块肿瘤组织由间隔和壁层组成骨皮质和骨膜广泛破坏，并侵犯软组织组织病理学特征出血腔由间隔壁隔开肿瘤组织，空腔 无腔壁而由其肉瘤组织自身形成提示其多态性和细胞的非典型性The 3rd Affiliated Hospital of Kunming Medical College44鉴别诊断临床上应与EWing瘤、纤维肉瘤和恶性纤维纳织细胞瘤鉴别在组织学上与动脉瘤样。对囊肿、骨巨细胞瘤、骨囊肿等相鉴别The 3rd Affiliated Hospital of Kunming Medical College45治疗及预后 因特别具有侵袭性而病程进展迅速，组织学表现恶性程度高治疗与标准骨肉瘤相同T

19、he 3rd Affiliated Hospital of Kunming Medical College46骨皮质旁型骨肉瘤（Parosteal Osteosaroma）源自骨旁和（或）源自骨表面的骨肉瘤它趋向于象牙质样致密改变病程缓慢，属低度恶性骨肉瘤发病率较典型骨肉瘤发病率低The 3rd Affiliated Hospital of Kunming Medical College47临床表现年龄及性别好发部位症状与体征The 3rd Affiliated Hospital of Kunming Medical College48年龄及性别一般在2050岁出现症状出现症状的潜伏期可能会很

20、长发病性别差异不明显。The 3rd Affiliated Hospital of Kunming Medical College49好发部位几乎全部发生在四肢长骨股骨最为常见（病例）其次为胫骨、肱骨、前臂骨罕见于躯干骨、手部骨和足部骨好发在干垢区，鲜有在骨干部The 3rd Affiliated Hospital of Kunming Medical College50症状与体征生长缓慢的无痛性肿块，病程较长疼痛出现较晚，呈中等程度肿瘤邻近关节时，关节功能受限The 3rd Affiliated Hospital of Kunming Medical College51线检查骨旁骨化肿块肿块

21、可沿干骷端环绕生长甚至侵袭宿主骨肿瘤与骨骼间有透光带相隔如不侵犯骨骼一般无骨膜反应软组织内可有大小不等的瘤骨The 3rd Affiliated Hospital of Kunming Medical College52CT、MRI断层检查逐层了解病变情况显示图像有助于对确治和预后评估了解宿主骨、髓腔、关节囊内的受侵袭情况The 3rd Affiliated Hospital of Kunming Medical College53病理学检查大体病理检查组织病理学特征The 3rd Affiliated Hospital of Kunming Medical College54大体病理检查肿瘤

22、呈球形或圆顶状，表面被假囊层包绕，层次分明肿瘤的结构坚硬，病灶内有大量的骨组织不成熟的骨化区充血，表面粗糙或在小梁骨方面有模糊的刻痕成熟和象牙质样骨部分呈现为白色，似正常的致密骨非骨化区域为白色，胶胨状的纤维或软骨肿瘤与宿主骨紧密融合，呈蘑菇样的部分向骨干过度生长肿块与皮质骨间有纤维层分开，有时则为肌胞膜分隔。The 3rd Affiliated Hospital of Kunming Medical College55组织病理学特征基本细胞由纤维基质所组成，含有骨样组织和小梁骨瘤细胞呈梭形细胞，数量很多有或多或少的胶原纤维成分小梁骨排列整齐，有一层丰富的骨母细胞围绕The 3rd Affil

23、iated Hospital of Kunming Medical College56鉴别诊断骨化性肌炎或异位骨化外生骨疣周围型软骨肉瘤骨膜骨肉瘤The 3rd Affiliated Hospital of Kunming Medical College57骨化性肌炎或异位骨化骨化常由损伤或反复的显微损伤修复所致数月成熟常见的部位为骨干病理学检查可鉴别。The 3rd Affiliated Hospital of Kunming Medical College58外生骨疣外生骨疣由外翻的皮质骨和干垢端的松质骨组成骨旁骨肉瘤是植人在增厚的骨皮质上病理学特征可鉴别。The 3rd Affiliat

24、ed Hospital of Kunming Medical College59周围型软骨肉瘤放射影像表现为表面突起像花篮，不透过放射线而且在软骨肉瘤侵人宿主骨之前，植人基部为一外生骨疣。解剖学方面及病理学方面差异明显The 3rd Affiliated Hospital of Kunming Medical College60骨膜骨肉瘤常侵犯骨于，骨膜被掀起呈透镜状放射线的透过性相当好，有薄而模糊的高密度影垂直于皮质骨大部分为成软骨组织并产生稀疏的和不成熟的骨样组织The 3rd Affiliated Hospital of Kunming Medical College61分期 骨旁骨肉瘤

25、期局限于骨旁间隙内期延伸到间室外期间室外延伸并高度恶变期The 3rd Affiliated Hospital of Kunming Medical College62治疗及预后 病程虽然缓慢，但可在任何时间内加速进展，并具有更大的侵袭性。及时而迅速适当地进行治疗，以免延误。边缘性切除术局部复发率较高广泛切除术肿瘤较大，侵犯骨髓腔/软组织、恶性度较高的病例，则应实施瘤段切除术截肢术的指征一般情况下，无截肢指征瘤体与主要血管粘连/肿瘤大/广泛的局部复发/恶性度高，可考虑行截肢术化疗只有在级的病例中，当肿瘤侵袭髓腔时应用本病对放疗不敏感The 3rd Affiliated Hospital of

26、Kunming Medical College63预后其病程缓慢，可多年无症状或仅有轻微症状肿瘤经反复切除复发，但始终无转移个别病例，病程进展快，恶性程度可达皿级，复发时恶性程度增加The 3rd Affiliated Hospital of Kunming Medical College64继发性骨肉瘤（Secondary osteosarco-ma）继发于Payet病其他良性病损（纤维异常增殖，良性软骨肿瘤）骨梗死慢性化脓性骨髓炎放射治疗后的肉瘤变The 3rd Affiliated Hospital of Kunming Medical College65继发性骨肉瘤常见于成人恶性程度依

27、其肿瘤来源不同而有所差异使用的治疗方法亦有所不同总体预后不良The 3rd Affiliated Hospital of Kunming Medical College66骨肉瘤病（Osteosarcomatosls）又称为多发性、多病灶性或多中心性骨肉瘤病不同部位、同时/相继出现在周身骨骼中的骨肉瘤病损发病率低The 3rd Affiliated Hospital of Kunming Medical College67临床表现 疼痛，分布非常弥散，不限于骨肉瘤的好发部位可见周身骨髓受侵犯The 3rd Affiliated Hospital of Kunming Medical Colle

28、ge68骨肉瘤病可分为三型：型：18岁以下发病，所有病灶同时出 现，恶性极高，预后差型：18岁以上发病，相继于个月内出现多骨性病灶型：任何年龄发病，诊断后24个月内/24个月以后不同时出现多骨性病灶The 3rd Affiliated Hospital of Kunming Medical College69影像学检查病灶呈均匀一致的硬化性或象牙样现象在大多数病灶中肿瘤均不超越骨皮质The 3rd Affiliated Hospital of Kunming Medical College70病理学检查 组织学检查多样化镜像，有不成熟的肉瘤细胞，有不同程度的类骨组织和肿瘤骨肿瘤的不同部位和不同部位的肿瘤似均相当一致The 3rd Affiliated Hospital of Kunming Medical College71治疗及预后病程迅速，预后差生存期短