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1 대한내과학회지 : 제 80 권부록 2 호 2011 비전형적영상소견을보이는악성심낭중피종 1 예 성균관대학교의과대학 1 삼성창원병원, 2 삼성서울병원순환기내과 천우정 1 박용환 1 오주현 1 장성아 2 송필상 2 류동열 2 강구현 1 A Case of Malignant Pericardial Mesothelioma with Atypical CT and MR Imaging Pattern Woo-Jung Chun 1, Yong-Whan Park 1, Ju-Hyun Oh 1, Seong-A Jang 2, Pil-Sang Song 2, Dong-Ryeol Ryu 2, and Gu-Hyun Kang 1 1 Division of Cardiology, Samsung Changwon Hospital, Sungkyunkwan University School of Medicine, Changwon; 2 Division of Cardiology, Seoul Samsung Hospital, Sungkyunkwan University School of Medicine, Seoul, Korea Primary malignant pericardial mesothelioma is a very rare and highly aggressive tumor with a poor prognosis. Here, we present the case of a 71-year-old man with symptoms of chest discomfort and exertional dyspnea starting 1 week prior to admission. We identified a pericardial mass using transthoracic echocardiography, but could not diagnose the patient using other multimodal imaging approaches and effusion cytology. Findings on contrast cardiac computed tomography (CT) and magnetic resonance imaging (MRI) were more consistent with angiosarcoma than malignant mesothelioma. Ultimately, the patient was diagnosed with malignant pericardial mesothelioma, based on immunohistochemical staining of the tumor tissue. In recent years, several cases have reported the efficacy of chemotherapy in malignant mesothelioma. Thus, we suggest that accurate diagnosis of pericardial tumors with pathology may have a profound impact on the final prognosis. (Korean J Med 2011;80:S161-S165) Keywords: Heart neoplasms; Mesothelioma; Pericardial effusion 서론심낭의원발성종양은폐암, 유방암, 흑색종, 림프종또는백혈병등이전이되어발생하는이차성종양보다드물게발생한다. 또한원발성종양은기형종, 섬유종, 혈관종, 지방종등의양성종양과, 중피종이나육종과같은악성종양이모 두나타나며, 원발성악성심낭종양은매우드물게나타나고있고한대규모의부검연구에서는그발생률을 % 로보고하고있다 [1]. 원발성악성심낭종양중가장흔한것이악성심낭중피종이지만역시절대적인발생률은매우적다고할수있고, 흉막의악성중피종과는달리석면노출병력과의관련성은적은것으로알려져있다 [2]. Received: Revised: Accepted: Correspondence to Gu-Hyun Kang, M.D. Department of Cardiology, Samsung Changwon Medical Center, Sungkyunkwan University School of Medicine, 50 Hapseong 2-dong, Masanhoewon-gu, Changwon , Korea Tel: , Fax: , iyedam@gmail.com -S161-

실제로우리나라에서처럼결핵의유병률이높은경우에는결핵성심막염및심낭삼출로오인되어진단이늦어진보고도있었다 [2]. 저자들은비특이적인영상검사소견을보이는심낭종괴를조직검사및면역조직화학염색을통해악성심낭중피종으로진단한 1예를경험하여문헌고찰과함께보고하는바이다. 증례 71세남자환자가내원 1주일전부터발생한흉부불편감과운동시호흡곤란을주소로응급실을내원하였다.

2 - The Korean Journal of Medicine: Vol. 80, Suppl. 2, 악성심낭중피종은협착성심막염, 심장눌림증 (cardiac tamponade) 또는관상동맥질환이나심근의침범으로인한심부전양상으로나타날수있으며 [3], 최근의발달된영상검사법들로도감별이어렵고, 심낭삼출액의세포병리검사에서악성세포가나오는경우도드물기 [4] 때문에진단하는데어려움이있다. 실제로우리나라에서처럼결핵의유병률이높은경우에는결핵성심막염및심낭삼출로오인되어진단이늦어진보고도있었다 [2]. 저자들은비특이적인영상검사소견을보이는심낭종괴를조직검사및면역조직화학염색을통해악성심낭중피종으로진단한 1예를경험하여문헌고찰과함께보고하는바이다. 증례 71세남자환자가내원 1주일전부터발생한흉부불편감과운동시호흡곤란을주소로응급실을내원하였다. 2년전타병원에서우관상동맥에관상동맥중재술을시행받은병력이있으며고혈압과당뇨병에대하여약물치료중인상태로내원시급성병색을보이지는않았으며혈압은 100/60 mmhg, 맥박수 60회 / 분, 호흡수 20회, 체온은 36.0, 동맥혈산소포화도는 95% 로측정되었다. 이학적검사에서는경정맥이확장되어있었고사지말단에경도의부종이있었으며심박동은규칙적이었고, 심잡음은들리지않았다. 심전도에서 Q파 는보이지않았으며정상동율동을보이고있었고, ST 분절이나 T파의변화는없었다. 단순흉부방사선촬영에서는심비대소견및좌심실음영뒤로미만성의석회화결절소견이보였다 (Fig. 1). 말초혈액검사에서백혈구 9,700/mm 3 ( 다핵구 67.8%), 혈색소 12.7 g/dl, 적혈구용적률 37.1%, 혈소판은 232,000/mm 3 이었으며혈청전해질검사에서나트륨 143 mmol/l, 칼륨 5.0 mmol/l이었으며혈청심근효소수치는 CK-MB 3.0 ng/ml, Troponin-I 0.03 ng/ml로정상수치를보였다. 내원당일시행한심장초음파상에서는다량의심낭삼출을동반하며우심실을압박하고있는종괴로인한협착성심막염이진단되었다 (Fig. 2). 내원 2일째검상돌기하접근법을통해심낭생검및심낭조루술 (pericardiostomy) 을시행하려하였으나수술실에서피부절개후심낭에수술기구가닿기만하여도심실빈맥 (ventricular tachycardia) 을포함한부정맥이발생하여수일후다시시도해보기로하고수술을마쳤다. 내원 3일째심낭천자를시행하여혈액성의삼출액 1 L를배액하였으며심낭삼출액을이용한세포병리검사상악성세포는발견되지않았다. 심낭천자이후환자의증상은호전을보였으며입원 8일째환자는 3차의료기관에서의검사를원하여삼성서울병원으로전원되었다. 삼성서울병원에서입원 2일째심장컴퓨터전산화단층촬영과자기공명영상검사를시행하였으며컴퓨터전산화단층 Figure 1. Posteroanterior chest radiography shows marked cardiomegaly and diffuse calcification in the left atrium (arrow). Figure 2. Subcostal views on transthoracic echocardiography show a moderate amount of pericardial effusion and massive compression of the right ventricle by the heteroechogenic pericardial mass (arrow). -S162-

- Woo-Jung Chun, et al. A case of malignant pericardial mesothelioma with atypical CT and MR imaging pattern - A B Figure 3.

with focal necrotic components (arrowhead) and mild enhancement of the dependent portion (pre, 47 HU post, 64 HU) was observed (arrow). Figure 4.

조직병리검사상에서방추세포 (spindle cell) 가증식되어있는병변이확인되었으며면역조직화학 (immunohistochemical) 염색에서는 vimentin 양성소견을보였고, 중피세포의표지자인 D2-40, cytokeratin AE1/3이양성으로확인되어 (Fig. 5) 악성중피종 (sarcomatoid type) 으로최종진단을내릴수있었다.

3 - Woo-Jung Chun, et al. A case of malignant pericardial mesothelioma with atypical CT and MR imaging pattern - A B Figure 3. Cardiac computed tomography, (A) non-enhanced imaging shows pericardial thickening and a calcified mass-like lesion, (B) enhanced imaging shows a heterogeneous mass that compressed the right heart with focal necrotic components (arrowhead) and mild enhancement of the dependent portion (pre, 47 HU post, 64 HU) was observed (arrow). Figure 4. Magnetic resonance imaging with perfusion scan (in different phases) shows enhancement of the pericardial mass in the arterial phase (arrow). 괴에대한조직검사를시행하였으며종괴에출혈성향이강하여수술을종료하였다. 조직병리검사상에서방추세포 (spindle cell) 가증식되어있는병변이확인되었으며면역조직화학 (immunohistochemical) 염색에서는 vimentin 양성소견을보였고, 중피세포의표지자인 D2-40, cytokeratin AE1/3이양성으로확인되어 (Fig. 5) 악성중피종 (sarcomatoid type) 으로최종진단을내릴수있었다. 이후환자는입원 18일째퇴원하여삼성서울병원혈액종양내과외래진료를보면서항암약물요법을권유받았으나더이상의치료를거부하고돌아와본원에서보존치료를시행중이다. 고찰 촬영에서는심장기저부의우측전외측으로심막의비후를동반한방사선투과성의종괴가있었고, 내부에조직괴사소견및석회화그리고부분적으로약간의조영증강소견을보였다 (Fig. 3). 자기공명영상에서는관류스캔 (perfusion scan) 에서종괴내부에조기조영증강이보이고있었다 (Fig. 4). 조기조영증강을보이는등이상의소견에서혈관육종 (angiosarcoma) 을의심하였으나정확한진단을위하여내원 10일째제5 늑간을통한우측전외측개흉술을시행하여종 악성심낭중피종의증상은서서히나타나며협착성심막염, 심장눌림증등의증상이흔하게발생한다. 또한다른종양과달리다량의심막삼출액보다는전반적인심막의비후를보이며협착성심막염 (constrictive pericarditis) 과같은임상양상을보이기때문에 [5,6] 종종진단이늦어지기도하며, 우리나라와같이결핵의유병률이높은지역에서는결핵성심낭염등으로오인되어항결핵제를투약받기도한다. 본증례에서는심장초음파검사상에서종괴병변이비교적명 -S163-

- 대한내과학회지 : 제 80 권부록 2 호 2011 - A B C D Figure 5.

(B) Immunohistochemical staining with vimentin was positive ( 400).

(D) Immunohistochemical staining with cytokeratin AE1/3 was focal positive ( 400).

더욱이영상검사상에서종괴내부에석회화를동반하는경우로는여러육종 (sarcoma) 에서보고된경우가 [7] 대부분이며, 흉곽의외상 (trauma) 후발생한출혈로인해이차적으로발생한경우의보고도있고 [8], 악성중피종에서석회화를동반한경우들은

4 - 대한내과학회지 : 제 80 권부록 2 호 A B C D Figure 5. (A) Hematoxylin and eosin-stained sections show a proliferation of spindle cells (arrow) ( 400). (B) Immunohistochemical staining with vimentin was positive ( 400). (C) Immunohistochemical staining with D2-40 was focal positive ( 400). (D) Immunohistochemical staining with cytokeratin AE1/3 was focal positive ( 400). 확히보였기때문에곧바로종양을의심할수있었지만, 컴퓨터전산화단층촬영및자기공명영상에서얻어진특징들, 즉종괴내부의석회화및조기조영증강소견으로는악성심낭중피종을의심하기어려웠다. 더욱이영상검사상에서종괴내부에석회화를동반하는경우로는여러육종 (sarcoma) 에서보고된경우가 [7] 대부분이며, 흉곽의외상 (trauma) 후발생한출혈로인해이차적으로발생한경우의보고도있고 [8], 악성중피종에서석회화를동반한경우들은 [9-11] 보고된증례의수가많지않다. 자기공명영상의관류 (perfusion) 영상에서보이는조영증강소견또한석회화와관련되어혈관육종 (angiosarcoma) 에가까운소견이었기때문에조직검사를통한면역조직화학검사를이용해확인하지 않았다면악성중피종을의심하기어려웠다. 악성심낭중피종은예후가매우불량하여평균생존기간이 6개월을넘지않는것으로알려져있으나최근에는일부항암약제를이용하여생존기간이 16개월에서 3년이상까지도늘어난증례들이보고되었다 [5,12]. 또한자기공명영상의관류스캔으로중피종의미세혈관구조를파악할수있고, 파악된미세혈관구조의분포율로서항암약물치료에따른예후를추정할수있다는연구도보고되었다 [13,14]. 비록본증례에서는환자가더이상의치료를거부하여그효과를확인할수없었으나이상의결과들을종합하면심막의종양이발견될경우에여러영상검사법및면역조직화학검사를시행하여정확한진단을내리는것이환자의예후에 -S164-

5 - 천우정외 6 인. 비전형적영상소견을보이는악성심낭중피종 1 예 - 큰영향을미칠수있는치료를결정하는데매우중요하다고할수있겠다. 요 악성심막중피종은예후가매우불량한것으로알려져있으며임상적으로다른양성질환과의감별이어려운경우도있고, 여러영상의학검사소견이다양하며심낭삼출액의세포병리에서암세포가나오는경우가적기때문에진단하기가쉽지않다. 하지만최근에는항암약물치료로효과를본증례들이보고되고있기때문에면역조직화학검사를시행하여정확한진단을내리는것이환자의예후에중요하다고할수있겠다. 약 중심단어 : 심장종양 ; 중피종 ; 심낭삼출 REFERENCES 1. Lagrotteria DD, Tsang B, Elavathil LJ, Tomlinson CW. A case of primary malignant pericardial mesothelioma. Can J Cardiol 2005; 21: Cho I, Chun EJ, Jeon KH, et al. A case of malignant pericardial mesothelioma misdiagnosed as tuberculosis pericarditis. Korean J Med 2009;76(Suppl 1):S81-S Suman S, Schofield P, Large S. Primary pericardial mesothelioma presenting as pericardial constriction: a case report. Heart 2004; 90:e4. 4. Thomason R, Schlegel W, Lucca M, Cummings S, Lee S. Primary malignant mesothelioma of the pericardium: case report and literature review. Tex Heart Inst J 1994;21: Butz T, Faber L, Langer C, et al. Primary malignant pericardial mesothelioma-a rare cause of pericardial effusion and consecutive constrictive pericarditis: a case report. J Med Case Reports 2009;3: Kainuma S, Masai T, Yamauchi T, Takeda K, Ito H, Sawa Y. Primary malignant pericardial mesothelioma presenting as pericardial constriction. Ann Thorac Cardiovasc Surg 2008; 14: Gladish GW, Sabloff BM, Munden RF, Truong MT, Erasmus JJ, Chasen MH. Primary thoracic sarcomas. Radiographics 2002; 22: Manhas AH, Martin RT, Reul GJ, Stainback RF. Heart failure due to a post-traumatic calcified pericardial hematoma. Tex Heart Inst J 2008;35: Raizon A, Schwartz A, Hix W, Rockoff SD. Calcification as a sign of sarcomatous degeneration of malignant pleural mesotheliomas: a new CT finding. J Comput Assist Tomogr 1996;20: Hillerdal G, Elmberger G. Malignant mediastinal tumor with bone formation: mesothelioma or sarcoma? J Thorac Oncol 2007;2: Quoix E, Chenard MP, Orion B, Lang-Averous G, Dopff- Kaissling A. A left pleural effusion with a calcified tumoral mass and left hemithoracic uptake on bone scan. Lung Cancer 2001; 32: Santos C, Montesinos J, Castaner E, Sole JM, Baga R. Primary pericardial mesothelioma. Lung Cancer 2008;60: Yamamuro M, Gerbaudo VH, Gill RR, Jacobson FL, Sugarbaker DJ, Hatabu H. Morphologic and functional imaging of malignant pleural mesothelioma. Eur J Radiol 2007;64: Giesel FL, Bischoff H, von Tengg-Kobligk H, et al. Dynamic contrast-enhanced MRI of malignant pleural mesothelioma: a feasibility study of noninvasive assessment, therapeutic follow-up, and possible predictor of improved outcome. Chest 2006;129: S165-

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