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1 다기관침범을동반한거대폐모세포종 이달의 X 선 기도개형연구실, 전북대학교의과대학내과학교실이태환, 이가영, 김소리, 민경훈, 박성주, 이흥범, 이양근, 이용철 A Case of Huge Pulmonary Blastoma With Multiorgan Invasion Tae Hwan Lee, M.D., Ka Young Lee, M.D., So Ri Kim, M.D., Kyung Hun Min, M.D., Seoung Ju Park, M.D., Heung Bum Lee, M.D., Yang Keun Rhee, M.D., Yong Chul Lee, M.D.,Ph.D. Department of Internal Medicine and Airway Remodeling Laboratory, Chonbuk National University Medical School, Jeonju, Republic of Korea A pulmonary blastoma is a rare malignant tumor of the lung that is composed of epithelial and mesenchymal elements and resembles the structure of an embryonic lung. Pulmonary blastomas have a very poor prognosis and make up 0.25 to 0.5 percent of all primary malignant lung tumors. A pulmonary blastoma usually manifests as a solitary parenchymal mass or nodule and multiple subpleural mass with effusion on chest X-ray and computed tomography. We encountered a very rare case of pulmonary blastoma in a 52 years old male. He complained of abdominal pain, fullness, and dyspnea. The radiology examination revealed a huge lung mass invading the mediastinum, heart, diaphragm, and liver. The percutaneous needle biopsies were performed, and this tumor was diagnosed as a pulmonary blastoma. We report a biopsy confirmed case of a huge pulmonary blastoma invading multiple organs. (Tuberc Respir Dis 2007; 62: ) Keywords: Pulmonary blastoma, Lung cancer, Metastasis. 증 환자 : 김OO, 52세, 남자주소 : 복부의팽만감과통증및호흡곤란현병력 : 환자는복부팽만감과통증및호흡곤란을주소로내원하였다. 이증상은 1달정도지속되었으나별다른치료없이지내던중점차그증상이악화되어응급실로내원하였고, 내원하여시행한단순흉부방사선촬영상우측폐하야와간에인접하여종괴가발견되었다. 과거력및가족력 : 5년전당뇨병과고혈압을진단받고복약중이었다. 진찰소견 : 호흡곤란당시활력징후는혈압 120/70 mmhg, 맥박 100회 / 분, 체온 36.5, 호흡수 25회 / 분 례 *Corresponding Author: Yong Chul Lee, M.D., Ph.D., Department of Internal Medicine, Chonbuk National University Medical School, San 2-20 Geumam-dong, Deokjin-gu, Jeonju, Jeonbuk , Republic of Korea Phone: ; Fax: ; leeyc@chonbuk.ac.kr Received: Nov Accepted: Dec 이었다. 환자는급성병색을보였으며, 결막과공막은특이소견없었다. 경부의임파선은촉지되지않았고, 흉부청진상우측하폐야에호흡음이감소되어있었고타진시우측에서둔탁하였다. 복부검진상경도의복부팽만외특이소견없었으며, 사지및신경학적검사에서이상소견은관찰되지않았다. 검사실소견 : 말초혈액검사상백혈구 9800/mm 3, 혈색소 14.0 g/dl, 혈소판 219,000/mm 3 이었다. 생화학검사상총빌리루빈 0.9 mg/dl, Aspartate aminotransferase 31 IU/L, Alanine aminotransferase 49 IU/L, 혈액요소질소 22.4 mg/dl, 크레아티닌 1.2 mg/dl 였으며, 전해질검사는정상범위였고, 호흡곤란이있어시행한동맥혈가스분석상 ph 7.45, PaCO mmhg, PaO mmhg, HCO meq/l, 산소포화도는 97.1% 였다. 방사선학적소견 : 내원시시행한단순흉부방사선에서우측폐야에 14cm 크기의종괴가관찰되고이는하방으로횡격막과간으로인접해보였다 (Figure 1). 이어시행한흉부전산화단층촬영에서는폐우하엽에약 cm 크기의종괴가관찰되었으며이종괴는하방으로횡격막과간, 심장을침범하고 149

TH Lee et al: A case of huge pulmonary blastoma with multiorgan

Chest radiographs show a huge mass on right lower lung field.

Photomicrograph of tissues obtained from percutaneous liver biopsy

with undifferenciated small round cells and loose spindle cells.

A CT scan shows a huge mass (14 12 11 cm) in the right lower lobe

(A) Enhanced axial view of CT scan (B) Coronal view of CT scan Figure

obtained from percutaneous liver biopsy shows positive reaction in

PET-CT scan shows unevenly hypermetabolic (p-suv=8.

diaphragm, and liver. 있었고, 경계는비교적분명하며조영증강시균일한조영증강을보였다.

2 TH Lee et al: A case of huge pulmonary blastoma with multiorgan invasion Figure 1. Chest radiographs show a huge mass on right lower lung field. (A) Posteroanterior view, (B) Lateral view of left side Figure 4. Photomicrograph of tissues obtained from percutaneous liver biopsy shows characteristic features of malignant stromal structure composed with undifferenciated small round cells and loose spindle cells. H&E stain ( 100). Figure 2. A CT scan shows a huge mass ( cm) in the right lower lobe invading diaphragm and liver. (A) Enhanced axial view of CT scan (B) Coronal view of CT scan Figure 5. Immunohistochemical stain for vimentin and CD99 of tissues obtained from percutaneous liver biopsy shows positive reaction in tumor cells ( 100). Figure 3. PET-CT scan shows unevenly hypermetabolic (p-suv=8.34) huge mass in the right lower lobe invading mediastinum, diaphragm, and liver. 있었고, 경계는비교적분명하며조영증강시균일한조영증강을보였다. 종격동과의지방선 (fat line) 은소실되었고간내상대정맥내에는혈전이관찰되었으며, 폐실질에전이성결절이나종격동내에의미있게커진림프절은보이지않았다 (Figure 2). PET-CT 상에서는폐우하엽에약 cm 크기의, 고르지않게대사가상승된종괴 (unevenly hypermetabolic, huge mass, p-suv=8.34) 가관찰되었으며이종괴는하방으로횡격막, 간을침범하고있었고, 종격동과도인접하여종격동과심장침범을의심하였다 (Figure 3). 병리소견 : 경피간바늘생검을시행한결과형태학적으로소원형세포 (small round cell) 과성근방추 150

Tuberculosis and Respiratory Diseases Vol. 62. No.2, Feb. 2007 Figure 6.

(A) H&E stain ( 100), (B) H&E stain ( 200).

3 Tuberculosis and Respiratory Diseases Vol. 62. No.2, Feb Figure 6. Photomicrograph of tissues obtained from percutaneous transthoracic needle biopsy shows a malignant stromal structure composed with undifferenciated and immature celluar component. (A) H&E stain ( 100), (B) H&E stain ( 200). 상세포 (loose spindle cell) 로구성된종양으로악성간질성종양 (malignant mesenchymal tumor) 소견을보였고 (Figure 4), 면역조직화학염색에서는 Desmin, S-100, Actin, CD 34, HMB 45, CD 31 에서는음성을보이면서 vimentin과 CD99에만양성소견이었다 (Figure 5). 이어서경피폐바늘생검을시행한결과미분화버팀질세포 (stromal cell) 들이특별한방향으로분화를보이지않고미성숙한세포의형태만을보이고있었다 (Figure 6). 두가지조직의생검소견을바탕으로하여폐모세포종을진단할수있었다. 치료및경과 : 진행된병기로화학항암치료예정이었으나치료를포기하고보존적치료만을위해호스피스병원으로전원되었다. 고찰폐모세포종은미성숙간엽세포와상피세포로구성되어져있는폐종양으로 1 매우드물게나타나며모든악성폐종양의약 % 정도의빈도를보인다 2. 대체로단일의큰종괴의형태를가지며주변조직과구분이잘되어있고, 폐주변부나폐상엽에주로호발하는것으로알려져있다 3. 일부환자에있어서특이증상이없는경우도있으나환자의약 60% 에서는증상이있고이중 80% 이상에서기침, 흉부통증, 호흡곤란및객혈등비특이적인증상을나타내며이러한증상들은폐종괴가기 관지나흉막을자극하여발생하게된다 4-6. 증상발생은종괴의크기와연관관계가있으며본환자의경우도횡격막하방으로침범된거대종괴로인한복부불편감을호소하였다. 종괴의크기는보고자에따라다 르나평균 6.9cm에서 10.1cm 정도였고, Koss et al. 등은 27cm 크기의종괴를보고하기도하였다 8. 폐모세포종의경우세포성분이적고방대한조직의괴사로인하여기관지내시경, 종격동경검사, 흉강경검사, 수술전가는바늘조직검사등의검사를하더라도수술전확진을내리기어렵고대부분수술후조직검사를통하여확진을내리게된다 9. 병리학적인면에서보면이상성폐모세포종 (biphasic pulmonary blastoma) 외에태아형폐선암종 (well diffenrentiated fetal adenocarcinoma), 폐막폐모세포종 (pleuropulmonary blastoma) 3가지로분류된다 10,11. 최근 WHO에서는이중이상성폐모세포종만을육종성분을갖는암종 (carcinoma with sarcomatoid elements) 으로분류하고, 태아형폐선암종은선암종의유형으로폐막폐모세포종은소아에서주로발병하는연부조직종양으로나누고있다 10. 폐모세포종은태아형상피조직과간엽조직이혼합된종양으로다양한형태의미성숙세포를가지므로상피나간엽조직세포를나타내는면역염색에서다양한양상으로나올수있고, 본증례의경우일부폐모세포종증례에서와같이 vimentin과 CD 99에서양성을보이면서특별한분화를보이지않는미성숙세포를보여폐모세포종이진 7 151

4 TH Lee et al: A case of huge pulmonary blastoma with multiorgan invasion 단되었다 10,11. 폐모세포종은대개폐문, 종격동, 림프절, 폐, 흉막, 흉벽, 횡격막및뇌로쉽게전이되며간, 흉외림프절, 심장으로의전이는드문것으로알려져있다 12. Zavala- Alarcou E et al 13 등이좌심방으로의침범을보고한바가있기는하나간, 심장, 종격동등을다발성으로직접침범한경우는보고된바가드물다. 이상성폐모세포종에서의전이성병변은이상성종양의형태를나타내기보다는대개균일하게분포되어있는단일형태나혼합형의조직형태를보이며그예후가불량하다 1. 폐모세포종의일반적인방사선학적인소견에대해서는아직까지명확하게보고된바는없지만, 대개단순흉부방사선학적소견은, 단일폐결절이나종괴의형태로, 보통폐상엽에나타나는것으로보고되었다 3,12. 흉부전산화단층촬영소견으로는역시단일폐결절혹은종괴의소견이나, 흉막하종괴, 흉수의소견으로서, 이러한소견들은원발성혹은전이성종괴에서비특이적으로볼수있는소견으로보고되었다 14. 본증례의경우단순흉부방사선에서우측폐야거의아래절반을채우는거대종괴로관찰되었다. 흉부전산화단층촬영에서역시폐우하엽에거대종괴가관찰되었고, 이종괴는하방으로횡격막과간, 심장을침범하고있었는데이는매우드문예라하겠다. 예후는전이의정도와종양의크기에따라다르지만, 일반적으로불량하며실제로환자의 2/3에서진단 2년이내에사망하고 5년생존률은 16%, 10년생존률은 8% 정도이다 6,8. 크기가크거나림프절등의전이가된경우예후는더욱불량하다. 치료는외과적으로절제하는것이가장좋으나타장기로의전이가있는경우보존적치료로방사선치료및화학요법을병행하기도한다 3,15. 하지만그효과는미미한것으로알려져있다. 본저자들은복부의팽만감과통증및호흡곤란을주소로내원한환자의단순흉부방사선검사에서우측폐야에 14cm 정도의거대종괴를볼수있었고, 이어촬영한흉부전산화단층촬영에서이종괴가폐우하엽의하방으로횡격막과간, 심장을침범하고있음을관찰하였다. 이에폐및간의바늘생검을통하여, 폐모세포종으로진단하였다. 폐및그하방의종 괴에대해경피간바늘생검과경피폐바늘생검을통하여간, 횡격막, 심장등다발성침범을동반한폐모세포종으로진단한 1예를경험하였기에보고한다. 요 폐모세포종은폐종양으로매우드물게나타나며드물게간, 흉외림프절, 심장으로전이된다고보고되고있다. 본저자들은복부의팽만감과통증및호흡곤란을주소로내원한환자에서간과폐의거대종괴를발견하였고, 조직검사를통하여간, 횡격막, 심장등다발성침범을동반한폐모세포종으로진단받은 1예를경험하였기에문헌고찰과함께보고하는바이다. 약 References 1. Francis D, Jacobsen M. Pulmonary blastoma. Curr Top Pathol 1983;73: Barnett N, Barnard WG. Some unusual thoracic tumors. Br J Surg 1945;32: Liman S T, Altinok T, Topcu S, Tastepe AI, Uzarc A, Demircan S,et al. Survival of biphasic pulmonary blastoma. Respir Med 2006;100: GP, Reed CE, Silvestri GA. Pulmonary blastoma: a rare lung malignancy. Lung Cancer : Di Lieto E, Baldi A, Vicidomini G, Di Marino MP, Baldi F. Pulmonary blastoma in adults. Minerva Chir 1997;52: Dogan R, Gungen Y, Ucanok K, Cetin G. Pulmonary blastoma. Hacettepe Med J 1989;22: Koss MN, Hochholzer L, O Leary T. Pulmonary blastomas. Cancer 1991;67: Jacobsen M, Francis D. Pulmonary blastoma: aclinicopathological study of eleven cases. Acta Pathol Microbiol Scand 1980;88: Novotny JE, Hurias CM. Resection and adjuvant chemotherapy of pulmonary blastoma: a case report. Cancer 1995;76: Travis WD, Brambilla E, Muller-Hermelink HK, Harris CC. Small cell carcinoma, and sarcomatoid carcinoma. In: World Health Organization Classification of Tumours. Pathology and genetics of tumours of the lung, pleura, thymus and heart. Lyon: IARC Press p , Dienemann D, Hartmann CA, Minck C. Pulmonary blastomas: immunohistochemical investigations of 152

5 Tuberculosis and Respiratory Diseases Vol. 62. No.2, Feb three cases. Pathol Res Pract 1989;184: Adluri RK, Boddu SR, Martin-Ucar A, Duffy JP, Beggs FD, Morgan WE. Pulmonary blastoma: a rare tumor with variable presentation. Eur J Cardiothoracic Surg 2006;29: Zavala-Alarcou E, Sudhakar S, Gonzoles LR, Patel R. Extension of a pulmonary blastoma into the left atrium. Mayo Clin Proc 2001;76: Lee HJ, Goo JM, Kim KW, Im JG, Kim JH. Pulmonary blastoma:radiologic findings in five patients. Clin Imaging 2004;28: Robert J, Pache JC, Seium Y, de Perrot M, Spiliopoulos A. Pulmonary blastoma: report of five cases and identification of clinical features suggestive of the disease. Eur J Cardiothorac Surg 2002:22:

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Kjtcs324( ).hwp 과오종과유사한폐상피모양혈관내피종 -1 예보고 - Pulmonary Epithelioid Hemangioendothelioma Mimicking Hamartoma -A case report- Epithelioid hemangioendothelioma, originating from the vascular endothelium, is a very rare and