NCCN软组织肉瘤临床实践指南2018.1版（8）
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目录
手术原则(SARC-D)
英文版
活检
●高度优选在治疗前进行活检以明确诊断和对肉瘤进行分级。活检应由有经验的外科医生（或放射科医生）进行，可通过开放性手术切除活检或用针穿刺活检完成。首选空心针穿刺活检；然而，有经验的外科医生可能考虑行开放性手术切取活检。对于深部、胸部、腹部或盆腔肉瘤可能需要行内镜或影像引导下针穿刺活检。
手术
●外科手术应切除肿瘤并获得安全边距。有时为了保留关键的神经血管结构、骨骼、关节等，切缘可能会靠近肿瘤。
●理想情况下，活检部位应该连同根治性手术的切除标本整块切除。切除平面应至始至终保持肉眼外观正常而不被肿瘤污染。如果肿瘤临近主要的血管或神经或使主要的血管或神经移位，去除动脉外膜或神经束膜而下层的神经血管结构肉眼未见受侵，则不需要切除这些血管或神经。
●根治性切除/整个解剖性切除不是常规必须的。
●手术夹子应放置在术野和其他相关结构的边缘做标识，以帮助未来可能需要做的放疗定位。如果使用闭式吸引引流，则引流应远离临近手术切口边缘的皮肤（对于再切除或需要行放疗的患者）。
手术切缘
●评估切除标本时，手术切缘应由外科医生和病理科医生共同记录。
●如果最终病理示手术切缘阳性（骨、神经或主要血管除外），而手术对功能没有显著影响，强烈推荐行手术再次切除以获得阴性切缘。
●对于软组织切缘距离肿瘤近，或者在骨、主要血管或主要神经的切缘呈镜下阳性的患者，应考虑行辅助放疗。
●对于非典型脂肪性肿瘤/高分化脂肪肉瘤（ALT/WDLS），大多数患者不需要行放疗。
●当切缘不确定时，推荐请放射科医生会诊。
► R0切除 - 无镜下残留病灶
► R1切除 - 镜下可见残留病灶
► R2切除 - 肉眼可见残留病灶
●浸润性组织学病变的患者（如粘液性纤维肉瘤、隆突性皮肤纤维肉瘤[DFSP]和血管肉瘤）应特别注意。
保留肢体手术
●对于肢体肉瘤，手术的目标应该是切除肿瘤的同时尽可能保留肢体的功能，如果可能，在肿瘤切除的安全边距范围内。
截肢
●在考虑截肢之前，应由擅长软组织肉瘤治疗的外科医生对患者进行评估。
●如果考虑通过截肢来治疗肢体肉瘤或如果预期肿瘤肉眼全部切除将导致肢体没有功能，在手术前应咨询患者的意愿。
●术前对肢体肉瘤患者进行康复评估（PT，OT）。继续康复锻炼，直至达到最大功能。
放射治疗指南(SARC-E)
SARC-E,1/3
肢体/躯干/头-颈软组织肉瘤的放射治疗指南
英文版
●Alektiar KM, et al. Impact of intensity-modulated radiation therapy on local control in primary soft-tissue sarcoma of the extremity. J Clin Oncol 2008;26:3440-3444;
●Kraybill WG, Harris J, Spiro IJ, et al. Phase II study of neoadjuvant chemotherapy and radiation therapy in the management of high-risk, high-grade, soft tissue sarcomas of the extremities and body wall: Radiation Therapy Oncology Group Trial 9514. J Clin Oncol 2006;24:619-625.
2.Haas RL，DeLaney TF，O'Sullivan B，et al：Radiotherapy for management ofextremity soft tissue sarcomas：why，when，and where？ Int J Radiat Oncol Biol Phys2012; 84：572-580。
3.这些指南倾向用于治疗成人患者。对于青少年和年轻成人肿瘤患者，转介NCCN青少年和年轻成人肿瘤指南。
4.外束放疗按1.8-2.0/f。
5.见手术原则中的“切缘”章节（SARC-D）。
6.总剂量应始终由正常组织耐受剂量决定。有数据表明，一些切缘阳性的患者，如那些低级别、分化良好的脂肪肉瘤患者以及在解剖学上固定的关键结构“计划中”的切缘阳性患者，经过术前放疗，术后没有补充剂量放疗也可能有好的效果。
7.也有数据表明阳性切缘的推量放疗并没有提高局部控制率。由于术后推量放疗没有明显获益，因此对于术后推量放疗应个体化决策并且谨慎考虑可能发生的毒副作用。(Al Yami, et al. Int J Radiat Oncol Biol Phys 2010;77:1191-1107; Pan, et al. J Surg Oncol 2014;110:817-822.)
8.放疗不能替代获得阴性切缘的根治性手术；再切除可能是必要的。
SARC-E,2/3
肢体/躯干/头-颈软组织肉瘤手术及术中放夹子标记后放疗
英文版
1.如果预期是R1或R2切除，提倡在复发高风险的区域放置夹子。当考虑行外放疗时，可使用调强适形放疗（IMRT）、螺旋断层放疗（托姆刀）和/或质子刀等精密治疗计划的放疗技术提高治疗比：
●Alektiar KM, et al. Impact of intensity-modulated radiation therapy on local control in primary soft-tissue sarcoma of the extremity. J Clin Oncol 2008;26:3440-3444;
●Kraybill WG, Harris J, Spiro IJ, et al. Phase II study of neoadjuvant chemotherapy and radiation therapy in the management of high-risk, high-grade, soft tissue sarcomas of the extremities and body wall: Radiation Therapy Oncology Group Trial 9514. J Clin Oncol 2006;24:619-625.
2.Haas RL，DeLaney TF，O'Sullivan B，et al：Radiotherapy for management ofextremity soft tissue sarcomas：why，when，and where？ Int J Radiat Oncol Biol Phys2012; 84：572-580。
3.这些指南倾向用于治疗成人患者。对于青少年和年轻成人肿瘤患者，转介NCCN青少年和年轻成人肿瘤指南。
4.外束放疗按1.8-2.0/f。
5.见手术原则中的“切缘”章节（SARC-D）。
6.总剂量应始终由正常组织耐受剂量决定。有数据表明，一些切缘阳性的患者，如那些低级别、分化良好的脂肪肉瘤患者以及在解剖学上固定的关键结构“计划中”的切缘阳性患者，经过术前放疗，术后没有补充剂量放疗也可能有好的效果。
8.放疗不能替代获得阴性切缘的根治性手术；再切除可能是必要的。
9.数据仍然限于使用高剂量率(HDR)近距离放疗治疗肉瘤。在获得更多数据之前，建议将HDR分割剂量限制为3-4 Gy。(Nag et al, Int J Radiat Oncol Biol Phys 2001;49:1033-1043, 2001).
SARC-E,3/3
腹膜后/腹腔内肉瘤的放射治疗指南
英文版
4.外束放疗按1.8-2.0/f。
5.见手术原则中的“切缘”章节（SARC-D）。
8.放疗不能替代获得阴性切缘的根治性手术；再切除可能是必要的。
10.Tzeng CW, Fiveash JB, Popple, et al. Preoperative radiation therapy with selective dose escalation to the margin at risk for retroperitoneal sarcoma. Cancer 2006;107:371-379.
11.Baldini EH, et al. Ann Surg Oncol 2015;22:2846-2852.
12.Baldini EH, et al. Int J Radiat Oncol Biol Phys 2015;92:602-612.
13.对于腹膜后/腹腔内肉瘤患者，不鼓励在手术以后行术后放疗。如果在手术切除前没有做过放疗，考虑随访并在局部复发的时候行可能的术前EBRT。见(SARC-D)。对于经过高度筛选的考虑术后EBRT增量的患者，鼓励术中在复发风险高或预期为R1/R2切除的区域放置夹子。当外放疗用于这些罕见的情况时，可使用IMRI、IGRT、和/或质子的高精治疗计划来提高治疗比。
►Trans-Atlantic RPS Working Group. Management of Primary Retroperitoneal Sarcoma (RPS) in the Adult: A Consensus Approach from the Trans-Atlantic RPS Working Group. Ann Surg Oncol 2015;22:256-263.
►Musat E, et al. Comparison of intensity-modulated postoperative radiotherapy with conventional postoperative radiotherapy for retroperitoneal sarcoma. Cancer Radiother 2004;8:255-261.
►Chung CS, et al. A comparison of 3D conformal proton therapy, intensity modulated proton therapy, and intensity modulated photon therapy for retroperitoneal sarcomas. Int J Radiat Oncol Biol Phys 2006;66(3S):116."
软组织肉瘤有效的全身治疗药物和方案（SARC-F）
SARC-F,1/6
非特异亚型软组织肉瘤有效的全身治疗药物和方案
英文版
非特异亚型软组织肉瘤有效的全身治疗药物和方案a,b,c | |||
非特异组织学亚型软组织肉瘤d,e | 胃肠间质瘤k | 硬纤维瘤（侵袭性纤维瘤病） | |
联合方案 | 单药方案 | •伊马替尼26,27 •舒尼替尼28 •瑞格菲尼29 伊马替尼、舒尼替尼和瑞格菲尼后疾病进展 •索拉非尼30-32 •尼罗替尼33,34 •达沙替尼35（针对D842V突变的患者） •帕唑帕尼36 •依维莫司+ TKIl
| •舒林酸37或其它非甾体抗炎药（NSAIDs），包括塞来昔布 •他莫昔芬±舒林酸38,39 •托瑞米芬40 •甲氨蝶呤和长春碱41 •低剂量干扰素42 •阿霉素为基础的方案43-45 •伊马替尼46,47 •索拉非尼48 •甲氨蝶呤和长春瑞滨49 •脂质体阿霉素50
|
•AD（多柔比星，达卡巴嗪）1-4 •AIM（多柔比星，异环磷酰胺，美司钠）3-6 •MAID（美司钠，阿霉素，异环磷酰胺，达卡巴嗪）3,4,7,8 •异环磷酰胺，表柔比星，美司钠9 •吉西他滨和多西他赛10,11 •吉西他滨和长春瑞滨f,12 •吉西他滨和达卡巴嗪13 •多柔比星和olaratumabg,14 | •多柔比星3,4,15 •异环磷酰胺9,16 •表柔比星17 •吉西他滨 •达卡巴嗪 •脂质体阿霉素18 •替莫唑胺f,19 •长春瑞滨f,20 •艾日布林f,h,21 •曲贝替定f,i,22,23,24 •帕唑帕尼f,j,25
|
非多形性横纹肌肉瘤
联合方案
•长春新碱，放线菌素D，环磷酰胺51
•长春新碱，多柔比星，环磷酰胺52
•长春新碱，阿霉素和环磷酰胺
与异环磷酰胺和依托泊苷交替53
•长春新碱，多柔比星，异环磷酰胺54
•环磷酰胺和拓扑替康55,56
•异环磷酰胺和阿霉素57
•异环磷酰胺和依托泊苷58
•伊立替康和长春新碱59,60
•长春新碱和放线菌素D61
•卡铂和依托泊苷62
•长春瑞滨f和低剂量环磷酰胺63
•长春新碱，伊立替康，替莫唑胺64
单药
•多柔比星65
•伊立替康56,66
•托泊替康67
•长春瑞滨f,68
•高剂量甲氨蝶呤m,69
•曲贝替定f,23,24,25
•软组织尤文肉瘤，见NCCN骨恶性肿瘤指南
注解：
a.在开始治疗之前，所有患者应由一个精于肉瘤专业知识和临床实践的多学科团队进行评估和管理。
b.对于子宫肉瘤，参见NCCN子宫肿瘤指南。
c.腺泡状软组织肉瘤 (ASPS)，高分化脂肪肉瘤/非典型脂肪性肿瘤和透明细胞肉瘤通常对细胞毒性化疗不敏感。
d.基于蒽环类的方案在新辅助和辅助治疗的情况下是首选。
e.方案适合于多形性横纹肌肉瘤。
f.仅推荐用于姑息治疗。
g.用于适合含蒽环类药物方案组织类型的软组织肉瘤。
h.治疗脂肪肉瘤是1类推荐。
i.治疗脂肪肉瘤和平滑肌肉瘤（L-型）是1类推荐。
j.帕唑帕尼不用于脂肪来源的肉瘤。
k.伊马替尼，舒尼替尼和瑞格菲尼是被FDA批准用于治疗胃肠间质瘤的三种药物。
l.考虑与依维莫司联合使用的TKI包括伊马替尼、舒尼替尼或瑞格菲尼。
m.对于一些中枢神经系统或脑膜受侵犯的患者，当放疗不适合时，高剂量甲氨蝶呤可能有用。
SARC-F,2/6
软组织肉瘤有效的全身治疗药物和方案
英文版
a.在开始治疗之前，所有患者应由一个精于肉瘤专业知识和临床实践的多学科团队进行评估和管理。
c.腺泡状软组织肉瘤 (ASPS)，高分化脂肪肉瘤/非典型脂肪性肿瘤和透明细胞肉瘤通常对细胞毒性化疗不敏感。
f.仅推荐用于姑息治疗。
SARC-F,3/6-6/6
软组织肉瘤有效的全身治疗药物和方案-参考文献
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分期（ST）
ST-1
组织病理学类型
英文版
脂肪细胞肿瘤
非典型脂肪瘤
分化良好的脂肪肉瘤
脂肪肉瘤，NOS
去分化脂肪肉瘤
粘液样/圆形细胞脂肪肉瘤
多形性脂肪肉瘤
成纤维细胞/成肌纤维细胞肿瘤
隆突性皮肤纤维肉瘤
纤维肉瘤性隆起性皮肤纤维肉瘤
色素沉着的隆突性皮肤纤维肉瘤
孤立性纤维瘤，恶性
炎性肌纤维母细胞瘤
低度肌纤维母细胞肉瘤
成人纤维肉瘤
黏液纤维肉瘤（以前称作粘液性恶性纤维组织细胞瘤
[粘液性MFH]）
低级别纤维粘液样肉瘤
硬化性上皮样纤维肉瘤
所谓的纤维组织细胞肿瘤
软组织巨细胞瘤
平滑肌肿瘤
平滑肌肉瘤（不含皮肤）
Pericytic（血管周围）肿瘤
恶性血管球瘤
骨骼肌肉肿瘤
胚胎性横纹肌肉瘤（包括葡萄状，间变性）
腺泡状横纹肌肉瘤（包括实性，间变性）
多形性横纹肌肉瘤
纺锤体细胞/硬化横纹肌肉瘤
血管肿瘤
网状血管内皮瘤
假肌源性（上皮样肉瘤样）血管内皮瘤
上皮样血管内皮瘤
软组织血管肉瘤
软骨-骨肿瘤
骨外骨肉瘤
胃肠间质瘤
胃肠间质瘤，恶性
神经鞘肿瘤
恶性外周神经鞘瘤
上皮样恶性外周神经鞘瘤
恶性蝾螈瘤
恶性粒细胞瘤
不确定分化的肿瘤
骨化性纤维粘液瘤，恶性
间质肉瘤，NOS
肌上皮癌
磷酸盐间充质肿瘤，恶性
滑膜肉瘤（NOS，梭形细胞，双相型）
上皮样肉瘤
腺泡状软组织肉瘤
软组织透明细胞肉瘤
骨外粘液样软骨肉瘤
骨外尤文肉瘤
促结缔组织增生性小圆细胞瘤
肾外横纹肌样瘤
血管周细胞上皮样细胞瘤（PEComa），NOS
内膜肉瘤
未分化/未分类的肉瘤
未分化（梭形细胞肉瘤，多形性肉瘤，
圆形细胞肉瘤，上皮样肉瘤，NOS）
经许可后使用，Fletcher CDM, Bridge JA, Hogendoorn P, Mertens F, eds. World Health Organization Classification of Tumours of Soft Tissue and Bone. Fourth Edition. Lyon: IARC;2013.
ST-2
头颈部软组织肉瘤的AJCC分期系统（2016年，第8版）
英文版
躯干和肢体软组织肉瘤的AJCC分期系统（2016年，第8版）
英文版
腹部和胸部内脏器官软组织肉瘤的AJCC分期系统（2016年，第8版）
英文版
胃肠间质瘤的AJCC分期系统（2016年，第8版）
英文版
腹膜后软组织肉瘤的AJCC分期系统（2016年，第8版）
英文版
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