胸部结节病综合影像学交流.ppt

结节病：影像学检查与诊断交流中国石油中心医院 影像科 杨景震2016-4制作（注：内有动画设置，浏览时请用放映模式）内 容一、本院近期两例结节病影像学回顾二、胸部结节病影像学诊断的交流1、“以假乱真”使你失去信心？2、影像学与病理相关性对比（深入认识影像特征）3、结节病影像检查与诊断的扩展4、结节病：PET-CT的应用？病例：1王某某，女性，63岁。影像号 4196045 患者5天前体检行胸部CT提示双肺多发微结节影，纵膈多发淋巴结肿大，双肺条索影，左肺上叶下舌段膨胀不全，左肺下叶支扩，右侧叶间胸膜结节状增厚。主诉：无明显咳嗽咳痰，轻度活动后气短，否认发热、胸痛、恶心呕吐、腹痛腹泻等症状。进一步行全身PET/CT提示纵膈多区、双侧肺门区多发淋巴结肿大，双肺多发小结节，代谢活性增高。左肺下叶局限性支气管扩张，双肺少许条索影，陈旧性改变。门诊以“纵膈淋巴结肿大”收入院。查血肿瘤指标：CA125、CA199、CA153、CA242、癌胚抗原、甲胎蛋白均未见异常。住院后行支气管镜透壁活检：纵隔第四、七组淋巴结提示：粉染纤维素样变性组织间见中性粒细胞及淋巴细胞浸润，并见少量支气管粘膜上皮。临床诊断：血清血管紧张素转换酶53.35U（升高）,结合影像、实验室检查及临床表现，考虑肺结节病可能大。PET图SUV值最大3.9，最小3.4延时SUV值：最大3.3，最小2.5病例：2李某某，男，45 岁，影像号：0004141878 主诉：咳嗽10天，余无特殊。2月前受凉后出现咳嗽，呈阵发性，无明显昼夜差异，无明显咳痰，无痰中带血等。伴有发热，最高体温达39，伴有畏寒，无寒战、盗汗、乏力等，就诊于当地社区医院，给予抗感染治疗，患者体温降至正常，但咳嗽持续存在。查胸部CT见双肺多发微结节影，纵隔多发淋巴结肿大，外院查肿瘤标记物、血常规均未见明显异常，门诊以“肺部阴影”收入院。入院后行支气管超声内镜检查，可见4R组淋巴结、7组淋巴结肿大，分别于上述两处超声内镜引导下穿刺，送病理活检。纵隔淋巴结穿刺活检结果提示：粉染纤维素样变性组织间见中性粒细胞及淋巴细胞浸润并见少量支气管粘膜上皮。中科院肿瘤医院病理学检查：符合肉芽肿性炎，需鉴别结核；与本院组织活检提示肉芽肿性病变一致。临床诊断：综合患者症状、影像及病理结果，诊断为肺结节病。本院病理图：炎性肉芽肿本院两例经临床、影像、病理诊断为结节病，回顾其影像特征：1、胸内结节病期；2、肺病变为微结节、小结节（胸膜下及支气管血管周围）及纤维灶；3、密集堆积模式的肺门及纵隔的淋巴结肿大，边界清楚，大小不等，CT增强均匀强化，PET呈高代谢的程度不一；4、例2，肿大淋巴结已经侵及腹部（右膈脚区）；左肺内较大的结 节高代谢；5、随着临床治疗，淋巴结与肺部病灶明显缩小。定义与标准：在美国胸科学会（ATS）与欧洲呼吸学会（ERS）结节病共识中，其定义包含了下列要点：原因不明；多系统受累，尤以肺、眼、皮肤受损居多；青年及中年发病；病理为非干酪性上皮样肉芽肿，排除其他已知原因；免疫特点：皮肤迟发型过敏反应受抑制，病变处的Th1细胞反应增强。文献认为，严格地讲，诊断应满足定义全部条件。在定义中，无一项内容可单独确诊该病，无论临床、影像还是病理，甚至有临床表现、影像支持及组织学证据时，结节病的诊断也非完全肯定的，无金标准。该病的诊断强调综合临床、影像学、病理，并排除其他可能致肉芽肿样改变的原因后，方可确诊，体现了定义中的“原因不明”，具有排他性。北京胸科医院张立群等认为对结节病诊断采取一定程度的从严是正确的，否则激素误治的后果是可怕的。结节病影像学检查及诊断认识上需要更新影像学的检查方法：胸片：传统方法；超声：多用于筛查；常规CT：常用的、基本胸部检查HRCT：是肺部重要的应用方法MRI：用此方法看什么？核医学与PET-CT：传统的67镓检查结节病缺乏特异性，现很少使用；那么PET-CT具有多大的价值？影像学表现：综合影像学评判，寻找特异性征象，做定性诊断；检出多系统病灶，即病变的定位、定量；搜寻式检查，例如无名热等，而检出结节病。注：Kveim实验已不再是结节病诊断的标准方法；肺泡灌洗液CD4/CD8比值+SACE是临床有效的方法 40多年前，Siltzbach 根据胸片对结节病分5期 0期：正常；期：仅有肺门淋巴结肿大；期：肺门淋巴结肿大+肺实质病变；期：仅有肺实质病变；期：纤维化。胸内结节病的分期是基于胸部X线片的表现，迄今基于HRCT的结节病分期尚未被确认。上述分期对判断预后及预测不经治疗而自然消退有一定价值。60-90%的期患者可发生自然消退，而在期仅有10-20%（原著Brett M,et al.薛蕴菁等主译：肺部高分辨CT诊断精要 2015.1版）国内1993年“结节病诊断及治疗方案”(第三次修订)，将胸部结节病分为3期。期：肺门淋巴结肿大,而肺部无异常；A 期：肺部弥漫性病变，同时有肺门淋巴结肿大；B 期：肺部弥漫性病变，不伴有肺门淋巴结肿大；期：肺纤维化。胸部结节病，影像表现特异性差，与一些疾病鉴别困难：-包括检出、评判需要综合影像学。胸部结节病影像：肺部及胸膜病变、淋巴结病变、心脏病变。胸部结节病CT影像可具有典型、不典型表现。胸部结节病PET-CT，评价意义不仅限于胸部。心脏结节病MRI应用，尤其结节病者出现传导阻滞或其他心脏不适。胸外结节病需要综合影像学：中枢神经、骨关节、脊柱、肝脾等。The great mimic:Pictorial review of differential diagnoses ofpulmonary sarcoidosisPoster No.:P-0077 Congress:ESTI 2015 Type:Educational PosterAuthors:J.P.A.Lopes,M.Simes,O.Fernandes,L.Figueiredo;Lisbon/PT DOI:10.1594/esti2015/P-0077Fig.1:(A)Axial unenhanced CT scan(mediastinal window)of a 50 year-old woman with sarcoidosis obtained at the level of the aortic arch demonstrates marked preaortic lymphadenopathies(arrow).(B)Axial unenhanced CT scan(mediastinal window)showssimilar findings(arrow)in a 27 year-old man with non-Hodgkin lymphoma.以假乱真：肺结节病鉴别诊断回顾淋巴结肿大：结节病与淋巴瘤可能对诊断失去信心？Fig.2:(A)Axial unenhanced CT scan(mediastinal window)of a 69 year-old woman with sarcoidosis demonstrates an eggshell-like calcification of a subcarinal lymphadenopathy(arrow).(B)This finding can also be observed in patients with silicosis,like in this 38 year-old man(arrow).蛋壳样钙化：结节病与矽肺Fig.3:Axial HRCT scans show conglomerate masses in the upper lobes,witharchitectural distortion and fibrotic changes(A)in a 45 year-old man with sarcoidosis and(B)in a 43 year-old man with silicosis.Perihilar distribution is more evident in the patientwith sarcoidosis.块状纤维化、结构破坏：结节病与矽肺Fig.4:(A)Axial HRCT scan shows a peripheral consolidation with air bronchogram inthe right upper lobe of a 28 year-old woman with sarcoidosis mimicking(B)organizingpneumonia represented here by a similar consolidation in the left lower lobe of a 63 year-old woman.Note the distinctive micronodular lesions in the patient with sarcoidosis.周围的肺实变伴空气支气管征：结节病与肺炎Fig.5:Axial HRCT scans show resembling findings of focal interlobular septal thickening(A)in the right upper lobe of a 53 year-old man with sarcoidosis and(B)in the right lowerlobe of a 59 year-old man with small cell carcinoma in the right hilum(not shown).局灶性不规则间隔增厚：结节病与小细胞肺癌Fig.6:Axial HRCT scans demonstrate irregular thin-walled cysts in the upper lobes of(A)a 39 year-old woman with sarcoidosis and(B)a 61 year-old man with LCH.Note also the micronodular parenchymal lesions in both patients.薄壁囊性模式：结节病与朗汉斯病Fig.7:(A)Axial HRCT scan of a 75 year-old woman with sarcoidosis shows Mosaic attenuation pattern and reticular densities similar to observed(B)in a 49 year-old woman with chronic extrinsic allergic alveolitis.马赛克模式和网状高密度：结节病与过敏性肺泡炎Fig.8:Axial HRCT scans show reticular pattern and honeycombing(A)in the upper lobes of a 58 year-old man with sarcoidosis and(B)in the lower lobes of a 55 year-old man with UIP.网状模式和蜂窝：结节病与普通型间质性肺炎Fig.9:(A)Axial HRCT scan shows extensive findings of ground glass attenuation,associated to traction bronchiectasis and reticulation in a 75 year-old woman with sarcoidosis.(B)Note the similarities with the axial HRCT scan of a 67 year-old woman with NSIP,although the ground glass attenuation areas are less extensive and have a preferential subpleural distribution.大量磨玻璃密度伴牵拉性支扩及网状结构：结节病与非特异性间质性肺炎Fig.10:Axial inspiratory HRCT scans demonstrate multiple areas of low attenuation forming a Mosaic pattern(A)in a 51 year-old woman with sarcoidosis and(B)in a 38 year-old man with bronchiolitis obliterans.多灶性低密度区并呈马赛克模式：结节病与闭塞性细支气管炎Fig.11:Marked parenchymal destruction with large bullae formation(A)in a 53 year-old man with stage IV sarcoidosis and(B)in a 36 year-old man with severe emphysematous disease.显著的肺实质破坏并肺大泡形成：结节病与严重的肺气肿以上的“以假乱真”病例浏览，使得结节病影像诊断很难接下来，我们结合文献复习结节病 影像表现与病理的对应关系结节病的组织学特征是非干酪样肉芽肿。肉芽肿中心有组织细胞、类上皮细胞和多核巨细胞构成的核，该核被淋巴细胞、散在分布的浆细胞和各种数量不等的纤维母细胞及外周的胶原包绕。微小肉芽肿结节的特点：与肺组织分界清楚、单个孤立于肺间隔内，或三五个、十几个成簇状沿淋巴管周围间隙分布。结节病的病理组织学结节病在影像学上的基本病变淋巴结肿大；微结节、实变、肿块、呼吸道病变（支气管狭窄或闭塞、肺不张、GGO、马赛克灌注、空气潴留）、纤维化（网状影、牵拉性支扩、纤维化块、囊性或蜂窝灶等）淋巴管周微结节常累及肺的4种结构：肺门旁支气管血管束周围间质；小叶中心（支气管血管束周围）间质；胸膜下间质、小叶间隔。即形成特征性HRCT影像所见（Brett M,et al著.薛蕴菁等主译：肺部高分辨CT诊断精要2015.1版）淋巴管周微结节HRCT上的4种表现1、支气管血管束周围结节4、小叶间隔结节 3、胸膜下结节另见结节病不典型征：不均肺内侵润（肉芽肿性病变在肺泡间隔和小血管周围的聚集，不伴肺泡炎）2、小叶中心结节Pulmonary Sarcoidosis:Typical and Atypical Manifestations at High-Resolution CT with Pathologic Correlation （RadioGraphics 2010;30:15671586 Published online 10.1148/rg.306105512）之一：The most common pattern is well-defined,bilateral,symmetric hilar and right paratracheal lymph node enlargement.Bilateral hilar lymph node enlargement,alone or in combination with mediastinal lymph node enlargement,occurs in an estimated 95%of patients affected with sarcoidosis(4,8,9).最典型的表现：95%淋巴结受累可表现为：边界清楚、双侧性、对称性肺门及右侧气管旁淋巴结肿大（注：形如希腊字母 ）或者表现为双侧肺门淋巴结肿大，伴或不伴纵隔淋巴结肿大。Figure 2.Typical(a,b)and atypical(c,d)radiologic findings of lymphadenopathy in four patients with sarcoidosis.(a)Axial contrast materialenhanced CT scan shows typical bilateral and symmetric hilar(arrows)and subcarinal(*)lymphadenopathy.(b)Axial unenhanced CT scan obtained at the level of the left pulmonary artery shows enlargement of right paratracheal and left hilar lymph nodes(arrows).Although the right hilum is not shown,it too was affected.(c)Axial unenhanced CT scan shows punctate calcifications of hilar lymph nodes(arrows),a pattern that also occurs in other chronic granulomatous diseases.(d)Axial contrast-enhanced CT scan shows bilateral eggshell-like calcifications of hilar and mediastinal lymph nodes(arrows),findings that warrant the inclusion of silicosis in the differential diagnosis in this case.典型的结节病不典型的结节病之二：之二：之三：之三：Figure 3.(a)Axial high-resolution CT scan of the right lung in a woman with pulmonary sarcoidosis shows the typical perilymphatic distribution of micronodules(arrow).(b)Photomicrograph of a lung biopsy specimen demonstrates numerous epithelioid granulomas(arrow)surrounding the bronchial walls and immediately beneath the normal bronchial epithelium(arrowheads).ABCT图 A：肺结节病：典型的淋巴管旁分布的微结节灶（箭）病理图 B：大量包绕支气管壁及直接位于正常支气管上皮下方的上皮样肉芽肿（长箭）之四：之四：Figure 4.Axial high-resolution CT scans obtained at the level of the upper lobes(a)and carina(b)in a patient with pulmonary sarcoidosis show a fibrotic-cicatricial pattern of disease,with multiple lesions in a peribronchovascular distribution.Characteristic features of chronic disease are depicted,including traction bronchiectasis,severe architectural distortion,volume loss,and interlobular septal thickening.Coalescent irregular masslike opacities(white arrows)and a calcified right lower paratracheal node(black arrow in b)also are seen.Mosaic attenuation,which is most visible in a,presumably results from airway distortion due to fibrosis.肺结节病：纤维瘢痕型，表现为支气管、血管周围分布的多发性病变。包括牵拉性的支扩，严重扭曲结构、体积缩小、肺小叶间隔增厚。其间伴不规则块样高密度影（白箭），另见钙化的气管旁淋巴结（黑箭）。小气道阻塞而导致的马赛克征。之五：之五：Figure 5.Typical manifestations of pulmonary sarcoidosis.(a)Axial high-resolution CT scan shows multiple micronodules with a peribronchovascular distribution in both lungs,predominantly in the upper and middle lobes.One cluster of nodules in the periphery of the left upper lobe(arrow)has coalesced to form a conglomeratelesion(macronodule).(b)Coronal reformatted image from high-resolution CT clearly shows upper-lobe predominance of the micronodules.(c)Low-magnification photomicrograph of a coronal slice from the lower part of the right upper lobe shows multiple confluent granulomas infiltrating the peribronchovascular(arrows)and subpleural(arrowheads)interstitium.肺结节病典型表现：轴位图：主要在两肺上叶和中叶并分布于支气管、血管周围的多发性微结节。左上叶周围多发性簇状结节凝聚成团（巨大结节）冠状位图：显示左上肺巨大结节更优越。低倍镜病理图：暗的部分为分布于血管、支气管周围（黄箭）、胸膜下间隙（箭头）融合的肉芽肿侵润。这种特征需记下：簇状多结节-融合巨大结节（星云征galaxy sign）病灶分布：支气管、血管周围及胸膜下间隙Figure 6.HRCT scan shows several large,ill-defined nodules and areas of consolidation resulting from the confluence of multiple parenchymal micronodules composed of numerous tiny granulomas in both lungs.Fine nodular opacities are seen around the large nodules(white arrows),and small low-attenuation spots that correspond to the spaces between partially coalescent small nodules are visible peripherally.This appearance has been termed the sarcoid galaxy sign.Distortion of the right major fissure is also seen(black arrow).Typical findings of sarcoid on HRCT,including hilar and mediastinal lympha-denopathy,bronchovasc-ular beading（串珠）and fissural nodularity.Axial HRCT image illustrating the galaxy sign(arrowhead).Note the widespread mediastinal and hilar lymphadenopathy andmore typical appearances of sarcoidosis including fissural nodularity.让我们再强化一下！Hawtin K.E.et al./Clinical Radiology 65(2010)642650之六：之六：Figure 7.Axial HRCT shows bilateral enlargement and peripheral calcification of mediastinal and hilar lymph nodes(arrows).Calcification also is visible within bilateral hilar parenchymal masses formed by multiple coalescent nodules with a peribronchovascular distribution.Histologic analysis of a specimen obtained at transbronchial biopsy of one of the masses showed sarcoid granulomas.Conglomerate masses and eggshell-like nodal calcifications also may be seen in silicosis,but they are more typically posteriorly situated within the upper lobes,not in hilar locations.之七：之七：双侧肺门、纵隔淋巴结肿大及淋巴结边缘钙化（箭）；血管、支气管周围分布的多发性融合的结节并由此形成的位于双侧肺门区的实质性肿块内也可见钙化。标本组织学为结节病性的肉芽肿。融合性肿块及蛋壳样的结状钙化也可见于矽肺，但后者更典型的位于上叶后部，而不是在肺门区。之八：之八：Figure 8.Sarcoidosis in a patient with a history of stage III primary cutaneous malignant melanoma.(a)Contrast-enhanced CT scans show pulmonary nodules(arrow)in subpleural(right)and fissural(left)regions.A video-assisted thoracoscopic surgical biopsy was performed.(b)Low-power photomicrograph obtained at histopathologic analysis shows a subpleural nodule that is darker in color because of anthracosis.(c)Photomicrograph obtained at higher power shows multiple nonnecrotic granulomas(arrows)expanding the interstitium that surrounds the subpleural nodule.结节病，有原发性皮肤恶性黑色素瘤期病史。增强CT显示肺结节（白箭）位于胸膜下和叶裂区。胸腔镜活检：低倍镜见胸膜下黑颜色的碳末结节；高倍镜下则显示胸膜下结节内多发性非坏死性肉芽肿（黄箭）。之九：之九：Figure9.Alveolar sarcoid pattern of airspace consolidation in pulmonary sarcoidosis.Axial high-resolution CTscan shows alveolar consolidation in the left upper lobe and patchy subpleural alveolar opacities in the right upper lobe.Architectural distortion and traction bronchiectasis,signs of fibrosis,also are visible,mainly in the right upper lobe.肺结节病：表现为肺气道实变模式肺泡肉样瘤型。CT显示左上叶肺泡实变、右上叶片状的胸膜下肺泡高密度。肺结构破坏、牵拉性支扩、纤维化征，以右上叶为主。Figures 10,11.GGO in pulmonary sarcoidosis.(10)HRCT depicts patchy GGO resulting from multiple coalescent micronodules in a peribronchovascular and subpleural distribution.Other changes depicted,all of which are indicative of fibrosis,include traction bronchiectasis,architectural distortion,cystic lesions,and septal thickening.(11a)HRCT shows a diffuse ground-glass pattern produced by multiple confluent micronodules,with associated bronchiectasis.(11b)Magnified HRCT of the right lung clearly depicts separate nodules in a subpleural(black arrow)and fissural(white arrow)distribution and along the bronchovascular bundles(arrowheads).(11c)High-power photomicrograph of a specimen obtained at wedge biopsy shows an accumulation of interstitial granulomas(white *),which causes a thickened appearance of the interalveolar septa,and acinar granulomas(black *),which form in the interstitium of the alveolar wall and protrude into the alveoli(arrowheads).之十：之十：肺结节病：磨玻璃影（GGO）图10：肺部片状GGO并与分布在支气管血管周围及胸膜下的多数密集的微结节灶融合；还可见纤维化及牵拉性支扩、病变结构破坏，囊性病变以及间隔增厚。图11a：弥漫性GGO模式，是由多数的微结节融合而成。图11b：HRCT放大图描述各自位于胸膜下（黑箭）、叶间裂（白箭）分布的结节，以及沿支气管血管束分布的结节（箭头）。图11c：活检标本高倍镜示，一个间质肉芽肿的积聚（白*），它引起肺泡内间隔增厚及腺泡肉芽肿（黑*），肉芽肿在肺泡壁的间隙形成并突入肺泡内（箭头）。图10图11a图11b图11c补丁状磨玻璃灶关于结节病CT增强扫描的问题结节病肿大的淋巴结在CT增强扫描图上呈均匀性强化，这种特点是结节病之病理决定的：结节病肉芽肿，文献名称2种：非坏死性肉芽肿（nonnecrotic granulomas），或称非干酪性肉芽肿（non-caseating granulomas）。淋巴结的中、轻度均匀强化并不能反映肉芽肿的进展与非活动，而这种活动与静止甚至自限性的病理变化能够在PET上评价（代谢成像）。增强扫描还可以通过观察肿大淋巴结边缘、有无融合以及内部的坏死而判断良恶，或结核病。对此，实际意义不及MRI平扫、PET.25例结节病均有淋巴结肿大：肿大淋巴结多密度均匀，轮廓清楚，无明显融合倾向，增强中度以上均匀强化。（张波绪等，医学影像学杂志 2016 年（26）：1.本院的例1淋巴结CT强化表现：各期均匀强化平扫结节病：影像学检查与诊断的扩展心脏结节病：适合于MRI检查 Semin Ultrasound CT MRI 35:215-224 C 2014心壁延时强化Balan A.et al./Clinical Radiology 65(2010)750e760延时12分钟增强扫描，心壁病变强化过去认为心脏结节病（CS）很少，但调查结果：美国至少25%结节病存在心脏受累，该病13-25%死于此；日本更高，有58-85%结节病死于心脏受侵。多是死亡后尸检发现。(A)Axial MRA shows multiple low-attenuation lesions(yellow arrows)in the spleen.(B)These multiple round lesions demonstrate enhancement(yellowarrow)even on late gadolinium sequences,and therefore are not cysts but rather represent multiple granulomas.Note also,midwall myocardial delayed enhancement in the basal anterolateral wall(white arrow)consistent with cardiac sarcoidosis.Semin Ultrasound CT MRI 35:215-224 C 2014图A：轴位MRA发现脾脏多发性低信号灶。图B为注射造影剂的后期，发现脾脏病灶有强化，因此说明并不是囊肿而是多发性肉芽肿。还需要注意到，心肌前下壁有延时强化，考虑有心脏结节病。神经系统结节病Semin Ultrasound CT MRI 35:215-224 C 2014 T1-weighted MR image of the brain before(A)and after contrast(B)administration illustrating enhancement along the optic nerve sheaths more conspicuous on the right(arrow).右图：MRI of the brain(axial and coronal)after contrast administration showing florid leptomeningeal enhancement,which is typical involvement of neurosarcoidosis(arrows).B图：沿右侧视神经鞘强化的结节灶脑的柔脑膜明显结节状强化，呈典型中枢神经系统结节病表现。Leptomeningeal sarcoid resulting in myelitis.A patient with systemic