胸膜间皮瘤92272

,单击此处编辑母版标题样式,单击此处编辑母版文本样式,第二级,第三级,第四级,第五级,2015-11-04,#,多模态成像在,恶性,胸膜间皮瘤特征、分类和分期的应用,大同三医院 王巧玲,概述,恶性胸膜间皮瘤是最常见的原发胸膜恶性肿瘤，占胸膜肿瘤的第二位，约,80%,的患者有石棉接触史，预后差，诊断后的中位,存活期为,9-17,个月。事实上，如果在疾病早期能及时诊断和实施针对的治疗方案，能够降低发病率和死亡率，提高生存率。国际间皮瘤研究组织根据总体存活率将疾病分为几个等级，分别是：原发肿瘤（,T），,淋巴结转移（,N）,和转移性疾病（,M），,放射科医生可以通过多种医学成像方法了解,MPM,的临床表现，将这些特征转化为相应的等级系统并提出相应的治疗方案。计算机断层扫描（,CT）,是用来评估,MPM,疾病特征的主要成像手段，能够有效地呈现原发肿瘤，胸内淋巴结病和胸腔外扩散的病变程度。然而，近年来诸如对胸腔的核磁共振成像,(,MR),以及带氟脱氧葡萄糖的正电子成像术,(,PET/CT),等成像技术作为,CT,成像的补充也用来分析,MPM,的患者。胸腔磁共振成像对于识别胸壁，胸腔纵隔膜和横膈膜的入侵非常有效，而,(,PET/CT),能够精确地显示胸腔内和胸腔外的淋巴结和肿瘤转移性疾病。,危险因素,石棉,第一，职业暴露石棉的人群，特别是直接暴露在蓝石棉下的采矿和磨矿工人。有作者曾对澳大利亚矿那些暴露在蓝石棉之下的人群进行深入细致的研究。那个地方曾经是历史上最可怕的工业灾难地之一。不仅矿工严重暴露在石棉之下，而且石棉残渣被用来取代草坪铺在学校的运动场和城镇的广场，结果导致恶性胸膜间皮瘤大爆发，很多年轻的患者是因为幼时在石棉废料上玩耍所致。,第二，间接职业暴露,的人群，即使用石棉产品的工人，如水管工人、木匠、防卫人员、石棉绝缘体安装工人等中也发现石棉相关疾病。,第三，环境暴露石棉的人群，,是指那些身处工业化国家而无意识地接触石棉者，他们占了恶性胸膜间皮瘤病例的,20%30%,。,危险因素,猿病,40,（,SV40,）,是一种,DNA,病毒，也被认为是恶性胸膜间皮瘤病因之一。这种病毒是存在于人类和啮齿动物细胞内的一种强力的瘤源性病毒，可以阻,断肿瘤抑制基因。在脑和骨的肿瘤、淋巴瘤和恶性胸膜间皮瘤里已经发现,SV40DNA,序列，在非典型间皮细胞增生和间皮非侵入性损害中也发现有该序列。有作者推测,35,至,50,年前,SV40,可能通过注射脊髓灰白质炎疫苗悄悄地传播给了人类。这种对,SV40,在恶性胸膜间皮瘤的发病机理中作用的假设已经成为争论的焦点，它的作用仍然有待证明。,危险因素,恶性肿瘤的放射治疗,例乳癌、肺癌等,流行病学及临床特征,起源于胸膜间皮细胞，可累及肺和胸壁，与石棉接触高度相关，潜伏期约,20-50,年，如果不进行治疗，,4-8,月死亡；石棉的接触时间和强度能增大,MPM,的致病性（石棉纤维致癌性与纤维的长宽比呈一定相关性，比率越高，致癌性越高）,通常发生于,50-70,岁，男：女,=4,：,1,，美国的年发生率,2500,人次,临床症状,:,非胸膜炎性的胸膜疼痛、呼吸困难,典型诊断：影像引导穿刺、手术活检，敏感性分别约,86%,、,94-100%,播散率,4%,、,22%,实验室检查,serum levels of soluble mesothelin-related protein (SMRP),是提高的，,METE,分析调查研究报告证明,SMRP,诊断,MPM,敏感性,64%,特异性,89%,，,CEA,、免疫组化渗出液、基因标记可以是阳性的,组织学类型,上皮型,55-65%,预后较好,肉瘤型,10-15%,侵袭性强,生存,6,月,混合型,20-35%,必须包含,10%,以上的上,皮和肉瘤成分,壁层胸膜多于脏层胸膜，右侧多于左侧，肿瘤可以融合呈胸膜斑块,;,尸检显示胸膜外转移的机率约,55%,分期,恶性胸膜间皮瘤的分期系统国际间皮瘤研究组,(IMIG),对恶性间皮瘤的分期标准,(1),T,原发瘤及其程度,Tla,肿瘤局限于壁层胸膜，包括纵隔和横膈胸膜；脏层胸膜未受累及。,Tlb,肿瘤累及壁层胸膜，包括纵隔和横膈胸膜；脏层胸膜也散在肿瘤病灶。,T2,肿瘤累及全部胸膜表面（壁层胸膜、纵膈胸膜、横膈胸膜、脏层胸膜），,横隔和,/,或脏层胸膜肿瘤互相融合，或者肿瘤从脏层胸膜侵犯下面的肺组织,T3,肿瘤为局部晚期，但有可能切除，肿瘤累及所有胸膜表面并累及筋膜（覆盖、,支持或连接肌肉或内脏器官的结缔组织薄膜）；肿瘤侵犯胸腔其他部位形,成单一可切除的肿块；,累及,心包。,T4,肿瘤为局部晚期、不可切除，累及所有胸膜表面，胸壁有肿瘤弥漫侵犯或形,成肿块，伴有或不伴有肋骨破坏；肿瘤直接穿破膈肌浸入腹膜；肿瘤直接蔓,延至对侧胸膜；肿瘤直接蔓延至一个或多个纵隔器官；肿瘤直接侵犯脊椎；,肿瘤侵犯心包膜的内层并伴有或不伴有心包积液，或者累及心肌。,恶性胸膜间皮瘤的分期系统国际间皮瘤研究组,(IMIG),对恶性间皮瘤的分期标准,(1),N,淋巴结,N0,无区域淋巴结转移,N1,转移至同侧气管肺或肺门淋巴结,N2,转移至纵隔或气管隆突（位于气管分叉下方）淋巴结,N3,转移至原发瘤对侧淋巴结,M,转移,M0,无远处转移,M1,有远处转移,影像特征,单侧胸腔积液、胸膜增厚、同侧容积减小、局部侵犯、淋巴结增大、远处转移，个别影像表现是非特异性的，出现一个以上要首要考虑，特别是有临床症状的患者,胸片,单侧胸腔积液，,30-80%,弥漫性胸膜增厚，胸膜结节发现比率分别约,60%,、,45-60%,肿瘤可延伸至叶间裂肺容积减小，同侧胸膜、纵膈胸膜转移、肋间隙变窄，区分骨化、钙化,石棉相关的胸膜斑,Figure 1.,Pleural effusion in a 47-year-old man with MPM.,(a),Posteroanterior radiograph shows a dependent right pleural effusion.,(b),Axial contrast material enhanced well-collimated CT image at the level of the mitral valve shows a moderate-sized right pleural effusion.,Figure 2.,Nodular pleural thickening in a 59-year-old man with MPM.,(a),Posteroanterior radiograph shows circumferential pleural thickening in the right hemithorax, with extension along the minor fissure (arrow).,(b),Axial contrast-enhanced well-collimated CT image at the level of the right pulmonary artery shows extensive nodular pleural thickening (arrows) in the right hemithorax. Note the ipsilateral volume loss.,(c),Coronal reformatted contrast-enhanced CT image at the level of the bronchus intermedius demonstrates extension of the tumor along the right minor interlobar fissure (arrow). The findings constitute a T2 tumor.,Figure 3.,Osteocartilaginous differentiation in a 54-year-old man with MPM.,(a),Posteroanterior radiograph shows extensive ossification of pleural disease in the left hemithorax. Nodularity seen along the lateral left hemithorax is consistent with chest wall invasion, and there is,ipsi,lateral volume loss. Calcified pleural plaques are seen in the right hemithorax (arrow).,(b),Axial nonenhanced well-collimated CT image of the inferior left hemithorax shows extensive tumor involvement with extension into the chest wall.,Figure 4.,Asbestos-related pleural disease in a 51-year-old man who subsequently developed MPM.,(a),Posteroanterior radiograph shows bilateral pleural plaques that result in a “shaggy” cardiac silhouette (white arrow) and ill-defined diaphragmatic contours (black arrow).,(b),Axial contrast-enhanced CT image at the level of the main pulmonary artery bifurcation shows extensive calcified and noncalcified pleural plaques secondary to long-standing asbestos exposure. Note the mediastinal pleural plaques (arrow), which are uncommonly seen.,CT,原始肿瘤延伸范围,局部发病情况,胸廓内淋巴结、纵膈侵犯、心包转移或胸腔外转移,肺内转移情况,单独评估肿瘤分期及治疗计划,胸膜局部增厚、环形或者大范围增厚超过,1cm,以上提示恶性胸膜疾病,区分钙化情况,Figure 5.,Mediastinal invasion in a 64-year-old woman with MPM. Axial contrast-enhanced well-collimated CT image at the level of the left ventricle shows a large right chest mass (white arrow) representing MPM that extends into the mediastinal fat, exerts mass effect on the right heart chambers, and occludes a right pulmonary vein (black arrow). A right pleural effusion is also seen. The loss of fat and tissue planes is consistent with mediastinal invasion. The mass constitutes a T4 tumor with invasion of mediastinal structures,；,Figure 6.,Mediastinal invasion in a 58-year-old man with MPM. Axial contrast-enhanced well-collimated CT image just inferior to the transverse thoracic aorta shows circumferential nodular pleural thickening in the right hemithorax. The tumor invades the mediastinum and surrounds the trachea and esophagus.,Figure 7.,Transmural pericardial invasion in a 66-year-old man with MPM and previous right extrapleural pneumonectomy. Axial contrast-enhanced well-collimated CT image at the level of the right pulmonary artery stump shows diffuse soft tissue surrounding the thoracic aorta and pulmonary arteries in the mediastinum (white arrows). Note the stump thrombosis of the right pulmonary artery (black arrow). A small left pleural effusion is also seen. Transmural involvement of the pericardium constitutes a T4 tumor and is unresectable.,Figure 8.,Focal chest wall invasion in a 54-year-old man with MPM. Axial contrast-enhanced CT image at the level of the right pulmonary artery shows pleural thickening in the anterior right hemithorax (arrowhead) and focal chest wall invasion (arrow). Note the small right pleural effusion and anterior mediastinal lymphadenopathy. Focal chest wall invasion with ipsilateral pleural involvement constitutes a T3 tumor and is resectable.,Figure 9. Invasion of the thoracic spine in a 61-year-old man with MPM. Coronal reformatted contrast-enhanced well-collimated CT image at the level of the descending thoracic aorta shows extensive pleural thickening in the right hemithorax (white arrow) and extension along the right major interlobar fissure (arrowhead). Note the invasion of the T2 vertebral body (black arrows), a finding that constitutes a T4 tumor and is unresectable.,Figure 10. Transdiaphragmatic extension in a 62-year-old man with MPM. Axial contrast-enhanced well-collimated CT image at the level of the right hemidiaphragm shows nodular pleural thickening in the right hemithorax and a right pleural effusion. There is complete encasement of the right hemidiaphragm and loss of the fat plane between the diaphragm and liver (arrows), findings suggestive of transdiaphragmatic extension of the tumor. The findings were confirmed at laparoscopy and represent unresectable T4 disease.,Figure 11. Intrathoracic lymphadenopathy in a 52-year-old man with MPM. Axial contrast-enhanced well-collimated CT image at the level of the transverse thoracic aorta shows extensive bilateral pleural thickening that is greater in the left hemithorax than in the right hemithorax. The enlarged right paratracheal (white arrow) and prevascular (black arrow) lymph nodes are consistent with nodal involvement and constitute N3 disease.,Figure 12. Pulmonary metastatic disease in a 59-year-old man with MPM. Axial contrast-enhanced well-collimated CT image just inferior to the pulmonary arteries shows extensive pleural thickening in the right hemithorax, with extension along the interlobar fissures. The thickening and nodularity of the interstitium and opacity in the right lung are consistent with lymphangitic carcinomatosis.,MRI,提供更精确的分期信息,检测胸壁、纵膈侵犯、和隔膜侵犯方面具有高度敏感性,单侧胸腔积液,T2,高信号,胸膜增厚，,T1,低信号，,T2,和质子像中等信号，增强呈显著强化,广泛应用于胸壁侵犯,(MRI:CT=69%:46%),、膈肌侵犯的诊断,(MRI:CT=82%:55%),Figure 13. MR imaging evaluation of a 61-year-old man with MPM. (a) Axial T1- weighted MR image at the level of the left pulmonary artery shows extensive pleural thickening that is isointense relative to muscle in the left hemithorax. (b) Axial T2-weighted MR image demonstrates diffuse hyperintensity of the thickened pleura relative to muscle. (c) Axial contrast-enhanced T1-weighted MR image shows diffuse enhancement of the thickened pleura in the left hemithorax.,Figure 14. Focal chest wall invasion in a 48-year-old man with MPM. Axial contrast-enhanced T1- weighted MR image at the level of the cavoatrial junction shows enhancing soft tissue in the right hemithorax, with focal invasion of the right anterior chest wall (arrowheads) and anterior mediastinal fat and pleural thickening in the posterior right hemithorax. The findings of invasion of the mediastinal fat and a single focus of chest wall invasion constitute a resectable T3 tumor.,Figure 15. Diaphragmatic invasion and transdiaphragmatic extension in a 59-year-old man with MPM. Sagittal T1-weighted MR image at the level of the right interlobar fissures shows encasement of the right hemidiaphragm and invasion of the anterior liver (black arrow). Diaphragmatic invasion and transdiaphragmatic extension of the tumor were confirmed at laparoscopy. Note the tumor extension along the right interlobar fissures (white arrows).,PET/CT,作为一种重要的辅助检查或许可用于,MPM,的诊断和分期，结合了,FDG,提供的代谢信息和,CT,提供的解剖信息,测量摄取值的大小，摄取值越大，生存时间越短,典型表现胸膜增厚区域呈异常高代谢浓聚,描述纵膈、胸壁侵犯、膈肌侵犯，更好的展示胸内外淋巴结转移情况,对于,、,期疾病,PET/CT,准确性,1.0,，,CT,分别为,0.77,、,0.75,，,PET,为,0.86,、,0.83,，,MRI,为,0.8,、,0.9,PET/CT,可因能识别最大,FDG,摄取位点、引导外科活检,评估治疗反应，检测疾病复发,Figure 16. FDG-avid pleural thickening in a 53-year-old man with MPM. Axial fused well-collimated PET/CT image at the level of the transverse thoracic aorta shows FDG-avid nodular pleural thickening in the left hemithorax (arrow), a finding that extends along the left interlobar fissure.,Figure 17. Focal invasion of the chest wall in a 53-year-old man with MPM. Axial fused well-collimated PET/CT image at the level of the T12 vertebral body shows FDG-avid pleural thickening in the right hemithorax and focal invasion of the right posterolateral chest wall (arrow). Focal invasion of the chest wall constitutes a T3 tumor and is resectable.,Figures 18, 19. (18) Transdiaphragmatic extension in a 64-year-old man with MPM. Axial fused well-collimated PET/CT image at the level of the T12 vertebral body shows FDG-avid pleural thickening in the inferior right hemithorax and irregularity of the surface contour of the liver, findings suggestive of transdiaphragmatic extension. Diaphragmatic involvement and transdiaphragmatic extension of the tumor were confirmed at laparoscopy. Note the FDG-avid lymph node (arrow) near the descending thoracic aorta. (19) Intrathoracic lymphadenopathy in a 69-year-old woman with MPM. Axial fused well-collimated PET/CT image at the level of the left atrium shows FDG-avid pleural nodules in the anterior right hemithorax, as well as FDG-avid paracardiac (black arrow) and right hilar (arrowhead) lymphadenopathy. An FDG-avid right axillary nodal metastasis (white arrow) is also seen.,Figure 20. Metastatic disease in a 53-year-old man with MPM. Coronal reformatted fused PET/CT image shows FDG-avid circumferential pleural thickening in the right hemithorax. A rounded focus of FDG uptake is seen in the right chest wall at the site of previous chest tube placement (arrow). A subtle focus of increased FDG uptake is seen in the left iliac wing (arrowhead). No lytic or sclerotic lesion was identified at CT, and the patient did not have symptoms referred to this area. PET/CT was used to direct tissue sampling of the bone lesion, which confirmed metastasis and excluded the patient from surgery.,Figure 21. PET/CT used to evaluate treatment response in a 57-year-old man with MPM. (a) Axial fused well-collimated PET/CT image of the inferior left hemithorax shows FDG-avid circumferential pleural thickening. (b) Axial fused well-collimated PET/CT image obtained after one cycle of cisplatin and pemetrexed therapy shows a significant interval decrease in FDG uptake in the tumor and a decrease in pleural thickening. A small pericardial effusion is seen.,Figure 22. PET/CT images in a 61-year-old man with recurrent MPM after left extrapleural pneumonectomy. (a) Axial fused well-collimated PET/CT image of the inferior left hemithorax shows FDG-avid soft tissue anteriorly, a finding consistent with recurrent MPM. (b) Axial fused well-collimated PET/CT image at the level of the left pulmonary artery shows an FDG-avid aortopulmonary window lymph node (white arrow) and an FDG-avid metastasis in the left posterior chest wall (black arrow).,鉴别诊断,胸膜转移瘤,最常见的胸膜恶性肿瘤，常见原发性肿瘤向胸膜转移有肺癌（,40%,）、乳腺癌（,20%,） 、淋巴瘤（,10%,） 、卵巢癌或胃癌（,5%,）,典型影像表现：胸腔积液、胸膜增厚、结节,特定的免疫学因子可鉴别,乳腺癌胸膜转移、肝多发转移,鉴别诊断,胸腺瘤,前纵隔最常见的原发性肿瘤，,a,期转移瘤胸腺瘤表现为胸膜增厚、胸膜结节或肿块，晚期可侵入纵膈脂肪、心脑血管结构,女，,70,岁，左侧胸痛二月余,鉴别诊断,局限性胸膜纤维瘤,恶性程度较低的肿瘤，起源于间皮下结缔组织和脏胸膜表面，,CT,显示小病灶一般较均匀，与胸膜呈钝角，大病灶呈锐角，随体位变化而变化,男，,45,岁，体检偶然发现,鉴别诊断,上皮性血管内皮瘤,罕见的可能与石棉接触有关的肺和肝脏的血管瘤，与,MPM,、胸膜转移瘤相似；,影像特征：包裹性胸腔积液、弥漫性小叶性胸膜增厚、胸膜肿块,免疫组分鉴别：主要依赖于内皮细胞产生血管特异性标记如因子,相关抗原、,CD31,、,CD34,等,治疗及预后,肿瘤因素如肿瘤的组织形态、肿瘤分期和患者因素如年龄、患者状态共同决定治疗方案。既往研究证明单一疗法（即手术，化疗，或放疗）对患者治疗效果有限。因此，目前的治疗方案注重多学科综合治疗，这种方式已表现出更好的治疗效果。,化疗,MPM,患者大多数在疾病晚期被诊断。因此，化疗仍然是治疗主要手段,顺铂为基础，培美曲塞作为一线药物用于不能手术切除的患者,放疗前辅助治疗,手术,、,期患者，胸膜切除术、胸膜剥脱术、胸膜外全肺切除术，最近研究表明这种治疗方式患者的生存分期与其他患者分期无统计学差异,术后复发率高，转移瘤少见,放疗,放射疗法是一种辅助性和姑息性疗法，因其具有肺损伤作用而不能使用根治剂量。虽然已有研究表明放射疗法能够减少肿瘤种植，但是目前不推荐使用预防性放疗。胸膜外全肺切除术后调强放射疗法（,IMRT,）可降低局部区域复发，在,IMRT,治疗前，应由肿瘤医师和外科医师共同决定手术切除范围，通常包括所有术前胸膜表面，同侧纵隔淋巴结，横隔膜后区域以及手术切口的深缘,Figure 23. CT images in a 62-year-old man with MPM who underwent extrapleural pneumonectomy and IMRT. (a) Axial contrast-enhanced well-collimated CT image at the level of the mitral valve shows postsurgical changes consistent with a left pneumonectomy. (b) On an axial nonenhanced CT image obtained for IMRT planning, contours have been drawn along the left hemithorax and include the preoperative pleural surfaces, retrocrural space, and deep margin of the surgical incision.,预后,MPM,患者总体预后不良，诊断后的中位生存期为,9-17,个月。然而，已经明确了某些因素影响存活时间。例如，上皮型,MPM,患者平均存活时间较肉瘤型,MPM,患者长。其他具有改善存活期的因素包括疾病局限于壁层胸膜，切除边缘肿瘤细胞检查阴性以及淋巴结未受累。导致预后不良或存活率降低的因素包括肿瘤为肉瘤型，胸痛，年龄大于,75,岁，男性，功能状态差以及体重减轻。,结论,MPM,是一种稀有的起源于胸膜间皮细胞的肿瘤，是最常见的胸膜原发肿瘤，肿瘤预后差，诊断后中位生存期是,9-17,个月，然而，早期诊断和治疗对改善存活率，降低发病率和死亡率是有用的，因此，放射科医师在运用,CT,、,MRI,、,PET/CT,对评估和分期,MPM,是相当重要的 ，,CT,保持基本的影像模式有效的评价原发肿瘤的范围，胸内淋巴结及胸外的转移情况，胸部,MRI,影像有效的补充了,CT,鉴别胸膜、纵膈及膈的侵袭情况，,PET/CT,对寻找胸腔内外的淋巴结及转移瘤是有用的。,