1、Bone TumorGao shichangThe First affiliated hospital of ChongQing Medical University1Epidemiology Incidence of bone sarcomas 骨肉瘤骨肉瘤 0.2%of all cancers 0.8 new cases per 100,000 population per year in North America and Europe Most common bone sarcomas#1:Osteosarcoma 骨肉瘤骨肉瘤#2:Chondrosarcoma 软骨肉瘤软骨肉瘤#3:
2、Ewing sarcoma 尤文肉瘤尤文肉瘤2 Age distribution is bimodal 双峰的双峰的First peak:Second decade of life Osteosarcoma predominates at this age Ewing sarcoma Second peak:60 years old Chondrosarcoma predominates Secondary osteosarcomasPagets osteosarcomaPostradiation osteosarcoma 放疗后骨放疗后骨肉瘤肉瘤3Gerneral principles Di
3、agnostic Evaluation An adequate history and physical examination are the first and most important steps in evaluating a patient with a musculoskeletal 肌肉骨骼的肌肉骨骼的 tumor.Patients present to the orthopaedic oncologist 骨骨科肿瘤医生科肿瘤医生 with pain,a mass,or with an abnormal radiographic finding detected durin
4、g the evaluation of an unrelated problem.4Gerneral principles The pain initially may be activity related,but the patient with a malignancy 恶性的恶性的 of bone often complains of progressive pain at rest and at night.Patients with benign bone tumors also may have activity-related pain if the lesion is lar
5、ge enough to weaken the bone5Gerneral principles Most notably osteoid osteoma 骨样骨瘤骨样骨瘤,may initially cause night pain.6Gerneral principles Age may be the most important information obtained in the history because most musculoskeletal neoplasms 肿瘤肿瘤,both benign and malignant,occur within specific age
6、 ranges.The physical examination should include evaluation of the patients general health,as well as a careful examination of the part in question.7Gerneral principles A mass should be measured,and its location,shape,consistency 一致性一致性,mobility,tenderness,local temperature,and change with position s
7、hould be noted.Atrophy of the surrounding musculature 肌肉肌肉组织组织 should be recorded,as well as neurological deficits 神经功能缺陷神经功能缺陷 and adequacy of circulation.8Gerneral principles Potential sites of lymph node metastases should be palpated 触及触及.All suspected musculoskeletal neoplasms should be evaluate
8、d initially with plain roentgenograms 平片平片.9Gerneral principles Often,the patients age and plain roentgenographic findings are sufficient to arrive at a specific diagnosis.10Gerneral principles Inactive lesions usually are well marginated 边边缘清晰的缘清晰的,often with a surrounding rim 边缘边缘 of reactive 反应的反
9、应的 bone formation.Aggressive lesions usually have a less well-defined zone of transition 过渡过渡 between the lesion and the host bone.Cortical expansion 皮质扩张皮质扩张 can be seen with aggressive benign lesions,but frank cortical destruction usually is a sign of malignancy.1112Gerneral principles Periosteal
10、骨膜的骨膜的 reactive new bone formation results when the tumor destroys cortex 皮质皮质 and may take the form of a Codmans triangle,“onion-skinning 透明片透明片,”or a“sunburst 炎爆炎爆 pattern.It usually is a sign of malignancy but may be present with infection or histiocytosis 组织细组织细胞增多胞增多.1314Gerneral principles Com
11、puted tomography(CT)is most helpful in assessing ossification 骨化骨化 and calcification 钙化钙化 and in evaluating the integrity 完整性完整性 of the cortex.CT of the lungs also is the most effective study to detect pulmonary metastases.15Gerneral principles CT also is the best imaging study to localize the nidus
12、 of an osteoid osteoma,to detect a thin rim of reactive bone around an aneurysmal bone cyst 动脉瘤样骨囊肿动脉瘤样骨囊肿.16Gerneral principles Technetium bone scans 锝骨扫描锝骨扫描 are indicated to detect skeletal metastases and to determine the presence of multiple lesions.Virtually 事实上事实上 all malignant neoplasms of bo
13、ne demonstrate increased uptake 摄取摄取 on technetium bone scans.1718Gerneral principles A normal bone scan is therefore very reassuring 放心的放心的;however,the converse 相相反的反的 statement 陈述陈述 is not true because most benign lesions of bone also demonstrate increased uptake.19Gerneral principles Magnetic res
14、onance imaging(MRI)has replaced CT as the study of choice to determine the size,extent,and anatomical relationships of both bone and soft tissue tumors.It is the most accurate technique for determining the limits of disease both within and outside bone.2021Gerneral principles MRI is not very useful
15、in differentiating benign from malignant lesions.In general,any soft tissue neoplasm deep to the fascia 筋膜筋膜 or larger than 5 cm in its greatest dimension 尺寸尺寸 should be considered highly likely to be a sarcoma.22Gerneral principles Ultrasonography is useful for distinguishing cystic 囊肿囊肿 from solid
16、 soft tissue.23Gerneral principles Blood and urine tests rarely lead to a diagnosis but can be useful in selected situations.Risk of wound-healing problems and infection have been shown to be significantly 显著地显著地 greater in patients whose serum albumin 血清白蛋白血清白蛋白 is less than 3.5 g/dl or whose total
17、 lymphocyte count is less than 1500/ml.24Gerneral principles A complete blood count may be helpful to rule out infection and leukemia 白血病白血病.The erythrocyte sedimentation rate 红细胞沉红细胞沉降率降率（ESR）usually is elevated in infection,metastatic carcinoma,and Ewing sarcoma.A prostate-specific antigen 前列腺特异抗原
18、前列腺特异抗原(PSA)should be ordered if prostate carcinoma is a possibility.25 Benign tumors are staged as follows:Stage 1 lesions are intracapsular 囊内的囊内的,usually asymptomatic 无症状的无症状的,and frequently incidental 偶然的偶然的 findings.Roentgenographic X线照相术线照相术 features include a well-defined margin with a thick
19、rim of reactive bone.There is no cortical destruction or expansion.STAGING 26Gerneral principles These lesions do not require treatment because they do not compromise 危害危害 the strength of the bone and usually resolve spontaneously 自发地自发地.27Gerneral principles Stage 2,active 1 The lesions also are in
20、tracapsular but are actively growing and therefore can cause symptoms or lead to pathological fracture 病病理性骨折理性骨折.2 They have well-defined margins on roentgenograms but may expand and thin the cortex.Usually they have only a very thin rim of reactive bone.3 Treatment usually consists of extended cur
21、ettage 扩大刮除术扩大刮除术.28Gerneral principles Stage 3,aggressive The lesions are extracapsular 囊外的囊外的.Their aggressive nature is apparent both clinically and roentgenographically.29Gerneral principles They usually have broken through the reactive bone and possibly the cortex.MRI may demonstrate a soft tis
22、sue mass,and metastases may be present in up to 5%of patients with these lesions.Treatment consists of extended curettage and marginal 边缘的边缘的 or even wide resection 切除切除术术,and local recurrences 复发复发 are common.30Gerneral principles Musculoskeletal sarcomas also can be staged according to the surgica
23、l staging system as described by Enneking et al.The system is based on the histological grade of the tumor,its local extent,and the presence or absence of metastases.31Gerneral principles Low-grade lesions are designated as stage I.These lesions are well-differentiated 高分化高分化的的,have few mitoses 有丝分裂
24、有丝分裂,and exhibit 展示展示 only moderate cytological atypia 细胞细胞学异型性学异型性.The risk for metastases is low(3 cm)and that do not involve major neurovascular 神经血管的神经血管的 structures.45Results Diagnostic accuracy rate 83%to 93%for musculoskeletal tumors Diagnostic accuracy is lower if the lesion is thought to be
25、 benign.46Open incisional BiopsyAdvantages Principal advantage relates to the larger size of the tissue sample obtained,reducing the risk of sampling error and allowing for more extensive histologic assessment as well as molecular diagnostic assessment 分子诊断评分子诊断评估估 when needed.Gold standard in achie
26、ving high diagnostic accuracy.47Disadvantages Principal disadvantage of open biopsy is potential for improper execution 不恰当的执不恰当的执行行 by surgeon.Potential adverse consequences of tissue contamination 污染污染/exposure during open biopsy.48Indications Many bone and less accessible deep soft tissue tumors
27、are best approached with open biopsy(see details below for incisional 切开切开的的 versus excisional biopsy 切除活检切除活检).Results Accuracy rates of 96%for extremity 肢体肢体 bone and soft tissue lesions following final analysis have been reported at major cancer centers.49Three Most Important Principles of Biopsy
28、 Incision Orientation 方向方向 Longitudinal,not transverse,incisions Longitudinal 长度长度,not transverse 非横向非横向,incisions 切口切口 Longitudinal,not transverse,incisions505152Treatment Surgical techniques Curettage:1 Many benign bone tumors are adequately treated by curettage.2 Compared with resection,curettage
29、 is associated with a higher rate of local recurrence;however,curettage often allows for a better functional result.53Gerneral principles Curettage is done by first making a large cortical window over the lesion.This window must be at least as large as the lesion itself.If the window is smaller than
30、 the lesion,the surgeon will inevitably 不可避免不可避免 leave residual 残余残余 tumor on the undersurface of the near cortex.54Gerneral principles “Extended”curettage includes the use of adjuvants 佐剂佐剂,such as liquid nitrogen 液氮液氮,phenol 苯酚苯酚,PMMA 聚甲基丙烯酸甲酯聚甲基丙烯酸甲酯,or thermal cautery 热烧灼热烧灼.55Gerneral principle
31、s The final issue that must be considered involves filling the cavity left after curettage.Options include autogenous bone graft 自体自体骨移植骨移植,allograft 同种异体移植同种异体移植,bone graft substitutes 骨移植替代物骨移植替代物,and bone cement 骨水泥骨水泥.5657 Calcaneus 根骨 Chondrosarcoma Two years laterGerneral principles Resection
32、and Reconstruction 重建重建 Tumor types Treatment Stage IA Local wide resection Stage IB Wide amputation 截肢截肢 Stage IIA Local radical 激进激进 resection Stage IIB Radical amputation58Gerneral principles Margins are determined by analysis of the surgical specimen 标本标本 to properly prepare for the resection.In
33、tralesional 病灶内病灶内 margins:Dissection passes 通过通过 within the lesion.Marginal 临界的临界的 margins:Dissection passes within reactive tissue contiguous 接触接触 with the lesion.59Gerneral principles Wide margins:Dissection is carried out through the normal tissue at a distance from the lesion.Radical margins:Al
34、l normal tissues of one or more compartments 隔室隔室 involved are removed from origin to insertion 插入插入.60616263Adjunctive Treatment 辅助治疗辅助治疗 Radiation therapy Most primary bone malignancies 恶性肿瘤恶性肿瘤 are relatively radioresistant 抗辐射抗辐射.Exceptions are the marrow 骨髓骨髓 cell tumors including multiple myel
35、oma 多发性骨髓瘤多发性骨髓瘤,lymphoma 淋巴瘤淋巴瘤,and Ewing sarcoma,which are each exquisitely 敏锐地敏锐地 sensitive.64Gerneral principles For most other bone tumors,radiation has a very limited role because local control is better achieved with surgery.65Gerneral principles Radiotherapy can be used to reduce the inciden
36、ce of local recurrence of malignant soft tissue tumors treated with marginal resection.Radiation also can be used for preoperative 术前术前 treatment of soft tissue sarcomas in the hopes of reducing the tumor volume and thus making the resection easier.66Gerneral principles Chemotherapy it refers to che
37、motherapy administered postoperatively 术后的术后的 to treat presumed 推推测的测的 micrometastases 微小转移微小转移.“Neoadjuvant 新辅助新辅助 chemotherapy refers to chemotherapy administered before surgical resection of the primary tumor.67AMPUTATION 截肢截肢 VERSUS LIMB SALVAGE 保肢保肢 Advances in diagnostic imaging,chemotherapy,r
38、adiation therapy,and surgical technique for resection and reconstruction now allow limb salvage to be a reasonable option for most patients with bone or soft tissue sarcomas.68 Preoperative radiation therapy for soft tissue sarcomas and neoadjuvant chemotherapy for bone sarcomas have helped surgeons
39、 to successfully resect some tumors that in the past would have been deemed 认为认为 unresectable.69 the choice between limb salvage and amputation must be made on the basis of the expectations and desires of the individual patient and the family.70 Four issues that must be considered whenever contempla
40、ting 考虑考虑 limb salvage instead of an amputation:71Will survival be affected by the treatment choice?How do the short-term and long-term morbidity 发病率发病率 compare?How will the function of a salvaged limb compare to that of a prosthesis 假体假体?Are there any psychosocial社会心理的社会心理的 consequences?72 With the
41、 use of multimodal 多峰的多峰的 treatment including surgery and chemotherapy,long-term survival for osteosarcoma patients has improved from approximately 20%to as high as 70%in some series.73 For osteosarcoma of the distal femur 远端肢骨远端肢骨,the rate of local recurrence after wide resection and limb salvage i
42、s approximately 5%to 10%.This is equivalent to 等同于等同于 the rate of local recurrence after a transfemoral amputation 单单侧经股骨截肢侧经股骨截肢 in this setting.74 With regard to overall patient survival,the most important technical aspect of the surgical procedure is the attainment 达到达到 of a wide margin regardles
43、s of whether this is achieved by amputation or local resection.75 A patient with a salvaged limb is much more likely to need multiple future operations for treatment of complications.These complications include periprosthetic fractures 假体周围骨折假体周围骨折,prosthetic loosening 假体松动假体松动 or dislocation 脱位脱位,n
44、onunion of the graft 移植移植-host junction,allograft fracture,leg length discrepancy 差异差异,and late infection.76 After initially successful limb salvage surgery,up to one third of the long-term survivors may ultimately 最终最终 require an amputation depending on the location of the tumor and the type of rec
45、onstruction.77 Future limb length inequality also must be considered.Although expandable prostheses 可扩展假体可扩展假体 and limb-lengthening 延长延长 procedures are available,the complications may outweigh 超超过过 the benefits especially in a child under 10 years of age.78 With regard to function,location of the tu
46、mor is the most important issue.Resection of a proximal 邻近的邻近的 femoral 股骨股骨的的 or pelvic 骨盆的骨盆的 lesion with local reconstruction generally provides better function than would be possible after a hip disarticulation 髋关节断离术髋关节断离术 or hemipelvectomy 偏侧骨盆切除术偏侧骨盆切除术.7980 Around the ankle 踝踝 and foot,howeve
47、r,large sarcomas frequently are treated with amputation followed by prosthetic fitting 装装配假体配假体.Treatment for sarcomas around the knee must be individualized 个性化个性化.81 Resection of an upper extremity lesion with limb salvage,even with the sacrifice 牺牲牺牲 of one or two major nerves,generally provides
48、better function than amputation and subsequent 随后的随后的 prosthetic fitting.8283Osteochondroma 骨软骨瘤骨软骨瘤 Osteochondromas are the most common of the benign bone tumors.They are probably developmental malformations 生长发育畸形生长发育畸形 rather than true neoplasms and are thought to originate 发生发生 within the perios
49、teum 骨膜骨膜 as small cartilaginous nodules 小软骨结节小软骨结节.84Osteochondroma The lesions consist of a bony mass,often in the form of a stalk 茎干茎干,produced by progressive endochondral 软骨内的软骨内的 ossification of a growing cartilaginous cap 软骨帽软骨帽.Their growth usually parallels that of the patient and usually ce
50、ases 停止 when skeletal maturity 骨成熟度骨成熟度 is reached.85Osteochondroma Osteochondromas may occur on any bone preformed in cartilage 软骨软骨 but usually are found on the metaphysis 干骺端干骺端 of a long bone near the physis 长骨体生长部长骨体生长部.They are seen most often on the distal femur 股骨远端股骨远端,the proximal tibia 胫骨
