软组织肿瘤患者的临床诊治要以患者获益为首要目标

姚阳

. 述评 Editorial .

软组织肿瘤患者的临床诊治要以患者获益为首要目标

姚阳

软组织肿瘤；肿瘤辅助疗法；肿瘤；外科手术；诊断

虽然，起源于多种细胞成分构成的恶性软组织肿瘤发病率较低，仅占成人恶性肿瘤的 0.73%～0.81%，15 岁以下儿童的 6.5%[1]。但是，在人体恶性肿瘤分类中最为复杂，国际通用的病理分类有 19 个组织类型和50 余种不同的亚型。与常见恶性肿瘤相比，国内外专门从事研究和临床软组织肿瘤治疗的科研人员和临床医师较少，学术活动、相关论文和书籍等也不够普及，临床医师可获得的继续教育资源相对匮乏。因此，临床上误诊、误治现象较为普遍，使得初诊可能治愈的部分病例，最终发生严重的不良后果；部分复发和转移的患者，失去了再次治愈的机会；随意性手术、非计划性手术，过度化、放疗的现象也较为普遍，甚至任意开展分子靶向治疗、细胞免疫治疗等。

毫不夸张地说，每一种类型的软组织肿瘤或者同种类型不同分化的恶性软组织肿瘤，都有其独特的生物学行为和转归，可以认为是一个独立的疾病[2]。如恶性脂肪细胞肿瘤就有 5 种不同的病理类型。由于各种类型软组织肿瘤分化不同，对化疗、放疗等敏感性差异也较大，其治疗原则和方式迥异[3]。不同年龄、不同部位、不同物理性状的软组织肿瘤，病理类型和预后也有一定的临床规律可循，如胚胎性横纹肌肉瘤好发于青少年头面部[4]，恶性神经鞘瘤易发生于中老年患者的四肢大神经分布处[5]，滑膜肉瘤多见于中青年的大关节附近[6]。

随着 PET-CT 技术和多维成像功能 MRI 的发展，软组织肿瘤的定位诊断以及其与周围血管神经的关系可获得充分显现，各种免疫组织化学技术及其基因和分子诊断使得软组织肿瘤的定性诊断和分类有了明显的进步，为选择合理的、个体化的综合治疗方法奠定了良好的基础。值得一提的是，影像技术和分子病理并不能够代替询问病史和物理检查。物理检查、影像学分析和病理检验三结合的临床诊断方法，对软组织肿瘤的诊断，缺一不可。

恶性软组织肿瘤是高度个体化肿瘤，不同类型、不同生物学行为的软组织肿瘤诊断和治疗方式存在很大的差异。每一个病例又各具临床特色，既没有明确细致的诊治规范，也缺乏大样本的循证医学的临床资料可以借鉴。目前，对于恶性软组织肿瘤需要多学科综合治疗的理念已被学界广泛接受[7]。不得不承认，手术是治愈绝大多数软组织肿瘤最主要的治愈性手段，抛开手术技术和经验，手术时机的把握显得尤为重要。手术时机决定了安全的外科边界[8]，循证医学资料表明，恶性软组织肿瘤安全的外科边界又是决定预后的第一要素[9]。过去在普遍认为软组织肿瘤对于化、放疗不敏感，“只有手术才是治愈软组织肿瘤的惟一手段”的错误概念误导下，反复手术、反复复发的现象较为普遍，殊不知对于化、放疗较为敏感的肿瘤，适当时机和剂量的新辅助化、放疗，让原本难以根治的体表软组织肿瘤达到 R0 ( 切缘无癌细胞，完整切除 ) 切除[10]。

近年来，随着 5-HT 受体和 NK-1 受体止吐药物、粒细胞集落刺激因子 ( granulocyte colony stimulating factor，GCSF )、血小板生成素 ( thrombopoietin，TPO ) 等细胞因子的问世，化疗药物毒副作用得到有效控制，术前大剂量、高密度新辅助化疗使得不少既往不能一期切除的软组织肿瘤成为可能，并且获得了安全的外科边界，显著提高了 5 年生存率[11]。精确放射治疗技术 ( SBRT ) 如：伽马刀、TOM 刀、质子重粒子刀相继进入临床，使得一部分失去手术机会的软组织肿瘤患者，其病情也获得了有效的控制，其中不少患者疗效达到了二次治愈[12]。ED743 治疗晚期脂肪肉瘤和平滑肌肉瘤已经获得欧盟和 FDA[13]批准入市。基因检测技术和靶向药物的快速发展，使一些骨与软组织肿瘤的疗效获得突破性进展[14]。培唑帕尼波美国 FDA 批准，用于治疗化疗失败的晚期软组织肉瘤 ( 除脂肪肉瘤和胃肠间质瘤 )，迪诺单抗治疗骨巨细胞瘤、克唑替尼治疗炎性肌纤维母细胞瘤等已获得不少成功案例[15]。国内生产的一类新药安罗替尼治疗软组织肉瘤已经完成了 II 期临床研究，对腺泡状软组织肉瘤和平滑肌肉瘤、滑膜肉瘤等显示了获得较好的疗效，为复发转移的软组织肿瘤的治疗增添了一丝曙光。

软组织肿瘤诊治工作越来越受到国内外学者广泛的重视，美国国立综合癌症网络 ( national comprehensive cancer network，NCCN ) 指南每年都出版一期软组织肿瘤的专家共识。由中国抗癌协会肉瘤专业委员会牵头编写的第一本《软组织肉瘤诊治中国专家共识》出版一年来，其临床实用性受到国内专家的一致好评。笔者呼吁开展软组织肿瘤临床诊治时，一切以患者能够获益为首要目的，务必作到“善战者先胜而后战”。

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( 本文编辑：李贵存 )

Patients’ benefit as the primary goal in clinical diagnosis and treatment of soft tissue tumors

YAO Yang.Department of Oncology, Shanghai sixth People’s Hospital, Shanghai, 201103, China

Although the incidence is low, the classification of malignant soft tissue tumors is the most complex in human malignant tumors. Compared with common malignant tumors, there are relatively fewer researchers engaging in the research and clinicians performing the treatment, and academic activities, related papers and books aren’t so popular. Therefore, clinical misdiagnosis and mistreatment are more common. Through the analysis of pathological types of soft tissue tumors, it is considered that localization diagnosis and qualitative diagnosis have laid a good foundation for the selection of reasonable and individualized comprehensive treatment methods. The appropriate time for surgery should be selected. With preoperative high-dose and high-density neoadjuvant chemotherapy and precise radiotherapy, combined with gene detection technology and targeted drug therapy, body surface soft tissue tumors that are hard to deal with before can be effectively cured. We are more conf dent in the treatment of recurrence and metastasis of soft tissue tumors, and furthermore, it’s benef cial for the patients.

Soft tissue neoplasms; Neoadjuvant therapy; Neoplasms; Surgical procedures, operative; Diagnosis

10.3969/j.issn.2095-252X.2017.02.001

R738.6

201103 上海第六人民医院肿瘤内科

2016-11-08 )