胸膜間皮瘤92272

1、大同三醫(yī)院大同三醫(yī)院 王巧玲王巧玲概述 惡性胸膜間皮瘤是最常見的原發(fā)胸膜惡性腫瘤，占胸膜腫瘤的第二位，約80%的患者有石棉接觸史，預后差，診斷后的中位存活期為9-17個月。事實上，如果在疾病早期能及時診斷和實施針對的治療方案，能夠降低發(fā)病率和死亡率，提高生存率。國際間皮瘤研究組織根據(jù)總體存活率將疾病分為幾個等級，分別是：原發(fā)腫瘤（T），淋巴結(jié)轉(zhuǎn)移（N）和轉(zhuǎn)移性疾?。∕），放射科醫(yī)生可以通過多種醫(yī)學成像方法了解MPM的臨床表現(xiàn)，將這些特征轉(zhuǎn)化為相應的等級系統(tǒng)并提出相應的治療方案。計算機斷層掃描（CT）是用來評估MPM疾病特征的主要成像手段，能夠有效地呈現(xiàn)原發(fā)腫瘤，胸內(nèi)淋巴結(jié)病和胸腔外擴散的病變程

2、度。然而，近年來諸如對胸腔的核磁共振成像(MR)以及帶氟脫氧葡萄糖的正電子成像術(PET/CT)等成像技術作為CT成像的補充也用來分析MPM的患者。胸腔磁共振成像對于識別胸壁，胸腔縱隔膜和橫膈膜的入侵非常有效，而(PET/CT)能夠精確地顯示胸腔內(nèi)和胸腔外的淋巴結(jié)和腫瘤轉(zhuǎn)移性疾病。危險因素石棉第一，職業(yè)暴露石棉的人群，特別是直接暴露在藍石棉下的采礦和磨礦工人。有作者曾對澳大利亞礦那些暴露在藍石棉之下的人群進行深入細致的研究。那個地方曾經(jīng)是歷史上最可怕的工業(yè)災難地之一。不僅礦工嚴重暴露在石棉之下，而且石棉殘渣被用來取代草坪鋪在學校的運動場和城鎮(zhèn)的廣場，結(jié)果導致惡性胸膜間皮瘤大爆發(fā)，很多年輕的患者

3、是因為幼時在石棉廢料上玩耍所致。第二，間接職業(yè)暴露的人群，即使用石棉產(chǎn)品的工人，如水管工人、木匠、防衛(wèi)人員、石棉絕緣體安裝工人等中也發(fā)現(xiàn)石棉相關疾病。第三，環(huán)境暴露石棉的人群，是指那些身處工業(yè)化國家而無意識地接觸石棉者，他們占了惡性胸膜間皮瘤病例的20%30%。危險因素猿病40（SV40） 是一種DNA病毒，也被認為是惡性胸膜間皮瘤病因之一。這種病毒是存在于人類和嚙齒動物細胞內(nèi)的一種強力的瘤源性病毒，可以阻斷腫瘤抑制基因。在腦和骨的腫瘤、淋巴瘤和惡性胸膜間皮瘤里已經(jīng)發(fā)現(xiàn)SV40DNA序列，在非典型間皮細胞增生和間皮非侵入性損害中也發(fā)現(xiàn)有該序列。有作者推測35至50年前SV40可能通過注射脊髓灰

4、白質(zhì)炎疫苗悄悄地傳播給了人類。這種對SV40在惡性胸膜間皮瘤的發(fā)病機理中作用的假設已經(jīng)成為爭論的焦點，它的作用仍然有待證明。危險因素惡性腫瘤的放射治療 例乳癌、肺癌等流行病學及臨床特征起源于胸膜間皮細胞，可累及肺和胸壁，與石棉接觸高度相關，潛伏期約20-50年，如果不進行治療，4-8月死亡；石棉的接觸時間和強度能增大MPM的致病性（石棉纖維致癌性與纖維的長寬比呈一定相關性，比率越高，致癌性越高）通常發(fā)生于50-70歲，男：女=4：1，美國的年發(fā)生率2500人次臨床癥狀:非胸膜炎性的胸膜疼痛、呼吸困難典型診斷：影像引導穿刺、手術活檢，敏感性分別約86%、94-100%,播散率4%、22%實驗室檢

5、查serumlevelsofsolublemesothelin-relatedprotein(SMRP)是提高的，METE分析調(diào)查研究報告證明SMRP診斷MPM敏感性64%,特異性89%，CEA、免疫組化滲出液、基因標記可以是陽性的組織學類型v 上皮型上皮型 55-65% 55-65% 預后較好預后較好v 肉瘤型肉瘤型 10-15% 10-15% 侵襲性強侵襲性強, ,生存生存66月月v 混合型混合型 20-35% 20-35% 必須包含必須包含10%10%以上的上以上的上 皮和肉瘤成分皮和肉瘤成分壁層胸膜多于臟層胸膜，右側(cè)多于左側(cè)，腫瘤可以融合呈胸膜斑塊;尸檢顯示胸膜外轉(zhuǎn)移的機率約55%分期

6、惡性胸膜間皮瘤的分期系統(tǒng)國際間皮瘤研究組(IMIG)對惡性間皮瘤的分期標準(1) T 原發(fā)瘤及其程度原發(fā)瘤及其程度TlaTla 腫瘤局限于壁層胸膜，包括縱隔和橫膈胸膜；臟層胸膜未受累及。腫瘤局限于壁層胸膜，包括縱隔和橫膈胸膜；臟層胸膜未受累及。TlbTlb 腫瘤累及壁層胸膜，包括縱隔和橫膈胸膜；臟層胸膜也散在腫瘤病灶。腫瘤累及壁層胸膜，包括縱隔和橫膈胸膜；臟層胸膜也散在腫瘤病灶。T2 T2 腫瘤累及全部胸膜表面（壁層胸膜、縱膈胸膜、橫膈胸膜、臟層胸膜），腫瘤累及全部胸膜表面（壁層胸膜、縱膈胸膜、橫膈胸膜、臟層胸膜）， 橫隔和橫隔和/ /或臟層胸膜腫瘤互相融合，或者腫瘤從臟層胸膜侵犯下面的肺組織

7、或臟層胸膜腫瘤互相融合，或者腫瘤從臟層胸膜侵犯下面的肺組織T3 T3 腫瘤為局部晚期，但有可能切除，腫瘤累及所有胸膜表面并累及筋膜（覆蓋、腫瘤為局部晚期，但有可能切除，腫瘤累及所有胸膜表面并累及筋膜（覆蓋、 支持或連接肌肉或內(nèi)臟器官的結(jié)締組織薄膜）；腫瘤侵犯胸腔其他部位形支持或連接肌肉或內(nèi)臟器官的結(jié)締組織薄膜）；腫瘤侵犯胸腔其他部位形 成單一可切除的腫塊；成單一可切除的腫塊；累及累及心包。心包。T4 T4 腫瘤為局部晚期、不可切除，累及所有胸膜表面，胸壁有腫瘤彌漫侵犯或形腫瘤為局部晚期、不可切除，累及所有胸膜表面，胸壁有腫瘤彌漫侵犯或形 成腫塊，伴有或不伴有肋骨破壞；腫瘤直接穿破膈肌浸入腹膜；

8、腫瘤直接蔓成腫塊，伴有或不伴有肋骨破壞；腫瘤直接穿破膈肌浸入腹膜；腫瘤直接蔓 延至對側(cè)胸膜；腫瘤直接蔓延至一個或多個縱隔器官；腫瘤直接侵犯脊椎；延至對側(cè)胸膜；腫瘤直接蔓延至一個或多個縱隔器官；腫瘤直接侵犯脊椎； 腫瘤侵犯心包膜的內(nèi)層并伴有或不伴有心包積液，或者累及心肌。腫瘤侵犯心包膜的內(nèi)層并伴有或不伴有心包積液，或者累及心肌。惡性胸膜間皮瘤的分期系統(tǒng)國際間皮瘤研究組(IMIG)對惡性間皮瘤的分期標準(1) N 淋巴結(jié) N0 無區(qū)域淋巴結(jié)轉(zhuǎn)移 N1 轉(zhuǎn)移至同側(cè)氣管肺或肺門淋巴結(jié) N2 轉(zhuǎn)移至縱隔或氣管隆突（位于氣管分叉下方）淋巴結(jié) N3 轉(zhuǎn)移至原發(fā)瘤對側(cè)淋巴結(jié) M 轉(zhuǎn)移 M0 無遠處轉(zhuǎn)移 M1

9、有遠處轉(zhuǎn)移影像特征 單側(cè)胸腔積液、胸膜增厚、同側(cè)容積減小、局部侵犯、淋巴結(jié)增大、遠處轉(zhuǎn)移，個別影像表現(xiàn)是非特異性的，出現(xiàn)一個以上要首要考慮，特別是有臨床癥狀的患者胸片單側(cè)胸腔積液，30-80%彌漫性胸膜增厚，胸膜結(jié)節(jié)發(fā)現(xiàn)比率分別約60%、45-60%腫瘤可延伸至葉間裂肺容積減小，同側(cè)胸膜、縱膈胸膜轉(zhuǎn)移、肋間隙變窄，區(qū)分骨化、鈣化 石棉相關的胸膜斑Figure 1. Pleural effusion in a 47-year-old man with MPM. (a) Posteroanterior radiograph shows a dependent right pleural effus

10、ion. (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) Posteroan-terior radiograph shows circumferential pleu-ral thickening in the righ

11、t hemithorax, with extension along the minor fissure (arrow). (b) Axial contrast-enhanced well-collimated CT image at the level of the right pulmo-nary artery shows extensive nodular pleural thickening (arrows) in the right hemithorax. Note the ipsilateral volume loss. (c) Coronal reformatted contra

12、st-enhanced CT image at the level of the bronchus intermedius demon-strates 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) Posteroante-rior radiograph shows ex

13、tensive ossification of pleural disease in the left hemithorax. Nodularity seen along the lateral left hemithorax is consis-tent with chest wall invasion, and there is ipsi-lateralvolumeloss.Calcifiedpleuralplaquesareseenintherighthemithorax(arrow).(b) Axialnonenhancedwell-collimatedCTimageoftheinfe

14、riorlefthemithoraxshowsextensivetumorinvolvementwithextensionintothechestwall.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

15、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 ar

16、e uncommonly seen.CT原始腫瘤延伸范圍局部發(fā)病情況胸廓內(nèi)淋巴結(jié)、縱膈侵犯、心包轉(zhuǎn)移或胸腔外轉(zhuǎn)移肺內(nèi)轉(zhuǎn)移情況單獨評估腫瘤分期及治療計劃胸膜局部增厚、環(huán)形或者大范圍增厚超過1cm以上提示惡性胸膜疾病區(qū)分鈣化情況 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 a

17、rrow) 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

18、 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 th

19、e 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 su

20、rrounding 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. Fo

21、cal 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 mediastin

22、al 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

23、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 exten

24、sion 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 betwee

25、n 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 i

26、mage 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 const

27、itute 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 a

28、nd nodularity of the interstitium and opacity in the right lung are consistent with lymphangitic carcinomatosis. MRI提供更精確的分期信息檢測胸壁、縱膈侵犯、和隔膜侵犯方面具有高度敏感性單側(cè)胸腔積液 ,T2高信號胸膜增厚，T1低信號，T2和質(zhì)子像中等信號，增強呈顯著強化廣泛應用于胸壁侵犯(MRI:CT=69%:46%) 、膈肌侵犯的診斷(MRI:CT=82%:55%)Figure 13. MR imaging evaluation of a 61-year-old man with

29、 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 co

30、ntrast-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 r

31、ight 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

32、 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 transdiaphr

33、agmatic extension of the tumor were confirmed at laparoscopy. Note the tumor extension along the right interlobar fissures (white arrows). PET/CT作為一種重要的輔助檢查或許可用于MPM的診斷和分期，結(jié)合了FDG提供的代謝信息和CT提供的解剖信息測量攝取值的大小，攝取值越大，生存時間越短典型表現(xiàn)胸膜增厚區(qū)域呈異常高代謝濃聚描述縱膈、胸壁侵犯、膈肌侵犯，更好的展示胸內(nèi)外淋巴結(jié)轉(zhuǎn)移情況對于、 期疾病PET/CT準確性1.0，CT分別為0.77、0.75，PE

34、T為0.86、0.83，MRI為0.8、0.9PET/CT可因能識別最大FDG攝取位點、引導外科活檢評估治療反應，檢測疾病復發(fā)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 find

35、ing 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 pos

36、terolateral 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 thickenin

37、g 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 descendin

38、g 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) ly

39、mphadenopathy. 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

40、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 t

41、issue 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 pleura

42、l 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-o

43、ld 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 pulmonar

44、y artery shows an FDG-avid aortopulmonary window lymph node (white arrow) and an FDG-avid metastasis in the left posterior chest wall (black arrow). 鑒別診斷胸膜轉(zhuǎn)移瘤最常見的胸膜惡性腫瘤，常見原發(fā)性腫瘤向胸膜轉(zhuǎn)移有肺癌（40%）、乳腺癌（20%） 、淋巴瘤（10%） 、卵巢癌或胃癌（5%）典型影像表現(xiàn)：胸腔積液、胸膜增厚、結(jié)節(jié)特定的免疫學因子可鑒別乳腺癌胸膜轉(zhuǎn)移、肝多發(fā)轉(zhuǎn)移鑒別診斷胸腺瘤 前縱隔最常見的原發(fā)性腫瘤， a期轉(zhuǎn)移瘤胸腺瘤表現(xiàn)為胸膜增厚、胸膜結(jié)節(jié)或腫塊，晚期可侵入縱膈脂肪、心腦血管結(jié)構(gòu)女，70歲，左側(cè)胸痛二月余鑒別診斷局限性胸膜纖維瘤 惡性程度較低的腫瘤，起源于間皮下結(jié)締組