NCCN骨肿瘤临床实践指南2017.2版（4）
好消息：
目前《指南解读》已经完成43个癌种NCCN临床实践指南及7个支持治疗指南（合计50个）的编译，后续我们将陆续再编译筛查、预防、降低风险指南以及老年肿瘤、青少年及年轻成人肿瘤指南并更新！有意获取中文指南电子版的同行，可加黄医生个人微信号30842121，我们将以PDF格式通过电子邮箱发送。考虑编译者团队在工作之余付出的大量辛苦工作，我们将向每位订制者收取360元（50个指南），敬请大家理解和支持！
目录
骨肿瘤管理原则（BONE-A）
活检
●在原发部位的任何手术或固定之前应进行活检诊断。
●活检最好在后面行根治性治疗的诊疗中心进行。
●活检位置是极其关键的。
●活检应该是芯针活检或手术活检。
●技术：穿刺活检或开放活检遵循相同原则。针穿刺活检不推荐用于颅底肿瘤。
●外科医生、肌肉骨骼放射科医生和骨科病理医生之间的适当沟通是非常重要的。
●分子检测和组织库可能需要新鲜组织。
●一般来说，没有遵循适当的活检程序可能引致不良的治疗效果。
手术
●广泛切除应实现手术切缘组织病理阴性。
●阴性的手术切缘有利于局部肿瘤的控制。
●局部肿瘤控制可通过保肢切除或截肢实现(根据患者制定个性化方案)。
●当可合理保全患肢功能时，保肢治疗是首选方案。
●最终病理评估应包括评估手术切缘和肿瘤的大小/尺寸。
实验室检查
●实验室检查如CBC、乳酸脱氢酶、碱性磷酸酶可能与骨肉瘤诊断、预后和治疗相关，应在患者做根治性治疗前进行，并在治疗和监测期间定期复查。
治疗
●化疗前应与患者说明对生育影响的问题。
●应由多学科小组的医生直接治疗骨肿瘤患者(１类推荐)。见TEAM-1。
长期随访和监测/监控
●患者应该有生存处方，安排一个多学科小组进行随访。
●对于长期幸存者，推荐进行长期随访以监测和治疗手术、放疗、和化疗的后期影像。
●根据临床需要，参见NCCN青少年和年轻成人（AYA，15-39岁）肿瘤指南。
骨肿瘤全身治疗药物（BONE-B）
软骨肉瘤
●传统型软骨肉瘤（1-3级）没有已知的标准化疗方案
●间叶型软骨肉瘤：遵循尤文肉瘤方案(2B类推荐)
●去分化型软骨肉瘤：遵循骨肉瘤方案（2B类推荐）
脊索瘤
►伊马替尼1,2,3
►伊马替尼联合顺铂4或西罗莫司5
►厄洛替尼6
►舒尼替尼7
►拉帕替尼用于EGFR阳性的脊索瘤8（2B类推荐）
►索拉非尼9,10
尤文肉瘤†
●一线治疗(主要治疗/新辅助治疗/辅助治疗)††
►VAC/IE
（长春新碱、多柔比星和环磷酰胺与异环磷酰胺和依托泊苷交替）11,12,†††
►VAI （长春新碱、多柔比星和异环磷酰胺）13,14
►VIDE （长春新碱、异环磷酰胺、多柔比星和依托泊苷）15
●初诊时为转移性疾病的初始治疗††
►VAdriaC（长春新碱、多柔比星和环磷酰胺）16
►VAC/IE11
►VAI13,14
►VIDE15
●二线治疗（复发/难治或转移性疾病）††††
►环磷酰胺和拓扑替康17-20
►伊立替康±替莫唑胺21-27
►异环磷酰胺（高剂量）±依托泊苷28,29
►异环磷酰胺、卡铂和依托泊苷30
►多西他赛和吉西他滨31
骨巨细胞瘤
►狄迪诺塞麦32-34
►α-干扰素 34-36
骨肉瘤†
●一线治疗 (主要治疗/新辅助治疗/辅助治疗或转移性疾病)
►顺铂和多柔比星37-39
►MAP（高剂量甲氨蝶呤、顺铂和多柔比星）40-41
►多柔比星、顺铂、异环磷酰胺和高剂量甲氨蝶呤42
►异环磷酰胺、顺铂和表柔比星43
●二线治疗（复发/难治或转移性疾病）
►多西他赛和吉西他滨31
►环磷酰胺和依托泊苷44
►磷酰胺和拓扑替康20
►吉西他滨45
►异环磷酰胺（高剂量）±依托泊苷28,46
►异环磷酰胺、卡铂和依托泊苷30
►高剂量甲氨蝶呤，依托泊苷和异环磷酰胺47
►153Sm-EDTMP用于复发或难治性疾病超过二线治疗的患者48
►Ra 22349-51
►索拉菲尼52
►索拉菲尼+依维莫司53
高级别未分化多形性肉瘤
●遵循骨肉瘤方案（2B类推荐）
参考文献：
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放射治疗原则（BONE-C）
●强烈鼓励患者在提供手术和全身治疗的同一个诊疗中心进行放疗。
●如有指征，应考虑专业技术，如：调强放疗（IMRT）；粒子束放疗（质子、碳离子或其他重离子）；立体定向放射外科；或分割的立体定向放疗，可在最大限度保护正常组织的同时给予高剂量治疗。
软骨肉瘤
●颅底肿瘤
►术后放疗或不可切除病变的放疗：>70Gy，使用专业技术
●颅外的部位
►可考虑给予术前放疗（19.8-50.4 Gy）（如果可能存在阳性切缘），然后行最终目标剂量为70 Gy（R1切除）1和72-78 Gy（R2切除）1的个体化术后放疗。
►可考虑行术后放疗 (60–70 Gy)，尤其是对于高级别/去分化/间叶型肿瘤距离切缘近或切缘阳性的患者。
►对于不可切除病灶考虑用专业技术行高剂量放疗。
脊索瘤
●颅底
►术后放疗（R1和R2切除）1或不可切除病变的放疗，70Gy或70Gy以上（总剂量将取决于正常组织的耐受剂量）。
►对于R0切除的患者考虑术后放疗。
●移动脊柱
►考虑术前放疗(19.8–50.4 Gy) 和术后放疗，总剂量70Gy（取决于正常组织的耐受剂量）。
尤文肉瘤
原发肿瘤的治疗
●根治性放疗
►应从12周的VAC/IE方案化疗或18周的VIDE化疗方案后开始
►放射野和剂量：
◊45Gy，初始肿瘤靶区（GTV1）+1-1.5cm(临床靶区，CTV1）+0.5-1cm（计划靶区，PTV1）
–肿瘤靶区（GTV1）定义为：治疗前骨和软组织病变的范围。如果肿瘤对化疗起效，正常组织恢复到自然位置，GTV1应除外化疗前延伸到腔隙的软组织的体积(如：化疗后肿瘤退缩，肺、肠或膀胱恢复到正常位置）
◊锥下野：覆盖原发骨肿瘤的范围+化疗后软组织（GTV2），总剂量55.8Gy+1-1.5cm（CTV2）+0.5-1cm（PTV2）
◊对于化疗后缩小<50%的患者，考虑增加调强剂量，总剂量为59.4Gy。
●术前放疗
►考虑用于临界切除的肿瘤，和巩固化疗同步进行。
►放射野和剂量：
◊ 初始GTV + 2 cm，36–45 Gy
●术后放疗
►应在术后60天开始放疗，与巩固化疗同步进行
►放射野和剂量：
◊R0切除：考虑用于组织学反应差的患者（即使切缘足够），45Gy，GTV2+1-1.5cm（CTV1）+0.5-1cm（PTV1）
◊R1切除：1 45 Gy ，GTV2 + 1–1.5 cm（CTV1） + 0.5–1 cm （PTV1）
◊R2 切除：1 45 Gy，GTV2+ 1–1.5 cm（CTV1 ）+ 0.5–1 cm（PTV1 ），随后对残留病灶进行锥下野放疗，合计总剂量55.8 Gy，GTV2 + 1–1.5 cm（ CTV2） + 0.5–1 cm（PTV2）
半胸照射
►应考虑用于胸壁原发合并大面积同侧胸膜受累的患者
►15–20 Gy (1.5 Gy/fx)，随后对原发部位进行锥下野放疗 (最终剂量取决于切缘)
转移性疾病的放疗
●在完成化疗/转移瘤切除术后进行全肺照射（3类推荐）
►15 Gy (1.5 Gy/fx) ，用于 <14岁的患者
►18 Gy ，用于>14岁的患者
●儿童肿瘤学组（COG）正在研究6岁前（12Gy）或6岁后（15Gy）的分组。
骨巨细胞瘤
转移性疾病的治疗
●考虑对序贯栓塞、狄迪诺塞麦、干扰素或长效干扰素（PEG IFN）治疗无反应的不可切除/进展/复发的肿瘤进行放疗 (50–60 Gy) 。
●在一些研究中已经注意到放疗后恶性转化的风险增加。
骨肉瘤
原发肿瘤的治疗
●对于切除后切缘阳性、次全切除或不可切除的患者应考虑进行放疗。
►术后放疗（R1或R2切除）：1 55Gy+对微观或肉眼病变进行9-13Gy调强放疗（对高危部位总剂量64-68Gy）。
►不切除病变： 60–70 Gy （总剂量取决于正常组织的耐受剂量）
转移性疾病的治疗
●考虑用153Sm-EDTMP和Radium 223
●考虑使用立体定向放射外科，尤其对于寡转移者。
注：
1.R0=无镜下病灶残留；R1=镜下肿瘤病灶残留；R2=肉眼肿瘤残留。
参考文献
软骨肉瘤
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Kawaguchi S, Weiss I, Lin PP, Huh WW, Lewis VO. Radiation therapy is associated with fewer recurrences in mesenchymal chondrosarcoma. Clin Orthop Relat Res 2014; 472(3): 856-864.
Rosenberg AE, Nielsen GP, Keel SB, et al. Chondrosarcoma of the base of the skull: a clinicopathologic study of 200 cases with emphasis on its distinction from chordoma. Am J Surg Pathol 1999;23(11):1370.
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脊索瘤
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尤文肉瘤
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骨巨细胞瘤
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组织学报告为混合型
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骨肉瘤
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分期（ST-1）
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