1087 대한방사선의학회지 1994 : 31(6) : 1087-1091 특발성폐섬유화증에동반된폐암 : 발생빈도및 CT 소견 1 이학졸 임정기 안중모 연경모 목적 : 특발성폐섬유화증을가진환자는폐암발생가능성이높다고알려져있다. 특발성폐섬유화증환자에서발생한폐암의발생빈도를파악하고 CT소견을정리하기위하여특발성폐섬유화증과폐암이병발한 19 명의환자를대상으로연구하였다. 대상및방법 :CT 및임상소견 (n =188), 혹은조직학적으로 (n =20) 특발성폐섬유화증으로진단된 208 명의환자중조직학적으로폐암이확진된 19명을대상으로하였다. 이들 19명의환자들은모두남자였고연령은 40-85세였으며평균연령은 66세였다. 1 2예에서일상적 C T를, 1 예에서고해상 CT를, 6 예에서는일상적 CT와고해상 CT를모두시행하였다. 이들환자에서폐암으 I CT유형, 종앙의위치, 그리고폐암의조직학적유형에대하여분석하였다. 결과 : 특발성폐섬유화증환자중폐암이동반된예는 9. 1%( 19 / 208) 이었으며, 종괴으 I CT유형은경계가불분명한경결양종괴로보이는경우가 11 예 (58 %) 로가장많았다. 폐암은대부분하엽에 ( 우측 10예, 좌측 5예 ) 위치하였고, 주로변연부에위치하였다 ( 12/ 19 ). 세포형은편평세포암이가장흔한유형이었다 ( 11 / 19 ). 결론 : 특발성폐섬유화즘에서페암이발생하는빈도는일반인에비하여월등히높았다. 동반된폐암의전형적인 CT소견은변언부에위치한, 경계가불운명한경걸앙종괴로나타나는경우가많아서가장섬유화가진행된부위에폐암발생이호발함을시사한다. 서 특발성폐섬유화증 (I PF) 을가진환자에서의폐암발생 율은정상인들에서보다 14 배나더높은것으로알려져있 다 (1). 특발성폐섬유화증에병발된폐암의흉부 X 선소견 에대하여는주로하엽에미만성의망상 (reticu l ar ), 선상 (linear) 음영을배경으로결절성병변으로나타난다고이 미보고된예가있으나 (2), 료응 - 특발성폐섬유화증과동반된 폐암의 CT소견에대해서는잘알려져있지않다. 본연구는특발성폐섬유화증 (I PF) 과병말한폐암의빈도와 CT 소견, 위치및세포유형을알아보기위하여 208 명의특발 성폐섬유화증환자중폐암으로확진된 19 명의환자를후 향적으로분석하였다. 대상및방법 최근 6 년동안 CT( 고해상흑은일상적 CT) 및임상소견 l 서울대학교의과대학방사선과학교실 본연구는 1994 년도서울대학교병원입상연구비의지원을받은것임 이논문은 1994 년 9월 2일접수하여 1 994 년 11 월 7일에채택되었음 으로진단되거나 ( n=1 88), 혹은조직학적으로진단된 (n= 20) 특발성폐섬유화증환자중에서조직학적으로폐암이 확진된 1 9 명을대상으로하였다. 병록지검토를통하여교 원혈관질환 (co llag e n vascular disease) 등특정질병에 의하여특발성폐섬유화증과비슷한소견을보이는환자 는대상에서제외되었다.208 명의환자중 67 명의환자는 폐기능검사를받았고그중 57 예에서제한성폐기능장애 를보였다. 폐암이진단된 1 9 예중 3 예는외과적적출술에 의하여폐암이진단되었고, 7예에서는객담세포병리검사 에서, 5예는경피적세침흡인술에의하여, 그리고 4예는경 기관지생검에의하여폐암이확진되었다. 이들환자는모 두남자였으며, 연령분포는 40 세와 85 세사이였고그평균 연령은 66 세였다. 환자중 12 명은일상적 CT 를시행하였 고, 1 명은고해상 CT 를시행하였으며 6 명은일상적 CT 와 고해상 CT 를모두시행하였다. 일상적 CT 는 CT / T 9800 scanner (General Electric Medical System, Milwaukee, Wisconsin) 로 10mm 두께및간격으로영상을얻였으며, 68.3% meglumine ioglicate 주사액 (Rayvist 300, Sche ring, Ber lin, Germany) 100 cc 를정맥주입하여조영증강 을하였다. 사진의 window width 빛 leve l을폐는 1500HU / -700HU 로하였고, 종격통은 400HU / -20HU 로하여
대한방사선의학회지 1994; 31( 6) : 1087-1091 촬영하였다. 고해상 CT 는같은기종으로 bone algori thm 을이용하여흉곽을절편두께 1.5mm, 간격 10mm 로영상 을얻였고조영증강은하지않았다. 고 찰 특발성폐섬유화증환자중폐암이동반된빈도를구하였고, 동반된폐암의 CT소견을그위치와형태에따라서경계가불분명한경결 (ill- defined consolidation) 병변, 결절 (nodule), 그리고기타소견으로나누어서고찰하였다. 폐종괴의위치는폐엽단위의분포를분석하였고단면영상 (axia l image) 에서는흉막으로부터 3cm를기준으로하여중심부와변연부의분포를분석하였다. 또한특발성폐섬유화증과동반된폐암의세포유형에대하여분석하였다. 특발성폐섬유화증은폐실질에진행성염증및섬유화가생기는질병이며예후는좋지않은것으로알려져있다 (4 ). 특발성폐섬유화증의자연경과는초기에폐포염으로시작되며, 이시기에는폐포벽및폐포강내의염증세포의침윤이주가되어 CT상에는마쇄유리상병변으로나타나며, 점차섬유화가진행되어말기가되면 CT상봉와상폐의양상이나타나게된다 (5). 폐의만성적섬유화로진행되는몇몇질환은종양의발생과관련이있음이발표된바있는데진행성전신성경화 결과특발성폐섬유화증환자에서폐암이동반된빈도는 208 명중 19명으로 9.1 % (1 9 /2 09) 이었다. 이때, 폐암의 CT소견은경계가불분명한경결양병변이 58% (1 1/19) 로가장많았고 (Fig. 1), 결절성병변이 26% (5/19), 기타소견이 1 6% ( 3/ 19) 이었다 (F ig. 2, 3, Table 1). 폐암의위치는우하엽, 화하엽, 우상엽이각각 53%(10/ 19), 26% (5/19), Table 1. CT Pattern 01 Lung Cancer associated with Idiopathic Pulmonary Fibrosis Pattern 01 lung cancer Number Percentage 111 delined consolidation 11 58 % Nodular 5 26% Undetermined 3 16 % n= 19 16% (3 / 19) 로우하엽에가장많이위치하였고, 변연부에 위치하는경우가 63%(12/ 19), 중심부에위치하는경우가 Table 2. Lobar Distribuion 01 Lung Cancer associated with 26% ( 5/ 1 9) 로변연부에위치하는경우가더많았다 (Table Idiopathic P비 monary Fibrosis 2, 3). 그러나여러엽에다발성으로존재하는예는없었다. 세포유형은편평세포암이 58% (1 1/19) 이었고, 선암이 21 % (4 / 19) 이었으며, 소세포암이 2 1% (4 / 19) 이였다 (Table 4). 특히 1 예에서는편평세포암과함께신경내분비세포암이함께동반되였다 (Fig.4). Location Number Percentage Right lower lobe 10 53 % Left lower lobe 5 26% Right upper lobe Diffuse 3 16 % 5% n=19 a Fig. 1. 70-year old man with dyspnea lor 3 years with idiopathic p미 monary librosis associated with squamous cell carcinoma 01 the lung with CT pattern 01 subpleural consolidation a. CT scan obtained at the level 01 the lower lung with a mediastinal window setting shows subpleural consolidation-like mass in right lower lobe(arrows) b. CT scan at the lower level than (a) with a lung window setting shows subpleural honeycombing in right lung and ground-glass opacity in left lung 1088
1089 - 이학종외 특발성폐섬유화증에동반된폐암 활짧 ~ 鳳 i - : : ii 순..-... 2a 2b 3 Fig. 2. 60-year old man with idiopathic pulmonary librosis associated with small cell carcinoma 01 the lung with a CT pattern 01 parenchymal nodule a. CT scan with a mediastinal window setting shows a lobulated mass in right lower lobe(arrows) with small amount 01 pleural effu sion b. CT scan with a lung window setting shows lung mass and peripheral microcystic change and ground-glass opacity Fig. 3. 56-year old man with presumed with idiopathic pulmonary librosis associated with bronchioloalveolar carcinoma. There are diffuse air space consolidation and subpleural honeycombing(arrows) Table 3. Axial Distribution 01 Lun g Cancer associated with Idiopathic Pulmonary Fibrosis Location Number Percentage Peripheral 12 63 % Central 5 26% Undetermined 2 11 % n= 19 Table 4. Histological Type 01 Lung Cancer associated with Idiopathic Pulmonary Fibrosis Cell type Number Percentage Squamous cell carcinoma 11 * 58 % Adenocarci nom a 4 21 % Small cell carcinoma 4 21% n= 19 * 1 case was synchronous double primary lung cancers 01 squamoll s and neu roendoc rine type 증 (progressive systemic sclerosis), 류마티스양간질성 섬유화증 (rheumatoid interstitial fi brosi s), 그리고특발 성섬유화성폐포염 (cryptogenic fibrosing al veolitis) 등 이그예이다 (6). 특발성섬유화성폐포염흑은특발성폐 섬유화증환자에서의폐암의발생빈도는 Turner - War wick 등 (1) 에의하면특발성폐섬유화증을가진 205명의환자중폐암이발생한경우는 20명으로 9.8% 이었고일반대중과비교하여 1 4.1배나높은것으로보고되였다. 그리고세포유형의분포는폐섬유화가없는폐암에서의분포와크게다르지않았다. 국한된폐의반흔에폐암이잘생긴다는사실은잘얄려져있다 (1, 11). 반흔이폐암의발생에어떠한영향을미치는가에대하여는정확하게알려져있지는않지만, 반흔이럼프관의폐색을일으켜서발암인자의국소적인농도를증가시키기때문일것이라는가능성 (11), 반흔이생긴부위와그렇지않은부위와의접합부위에생긴상피세포의화생 (metap l asia) 와과형성 (hyperp las i a) 등 (7, 12, 13) 이발암의요인으로보고된바있다. 이러한기전은미만성의간질성섬유화의경우에도적 용될수있다. H addad 등 (2) 의연구에의하면특발성폐섬유화증이있었던 8예에서모두종말기강 (terminal air space) 에비정형적인상피세포의증식이있었고, 이들중 3예에서폐암이동반되었으며이들은모두심한섬유화가있는부위에서발생하였고조직검사상이형성의세기관지폐포상피 (atypical or dysplastic bronchiolar a l ve이 ar epithelium) 에서폐암으로이행하고있었다 (2, 13). 미만성폐간질섬유화에서폐암이발생하는근본적인원인은알려지지않았지만미만성의간질성섬유화에서생기
대한방사선의학회지 1994 ; 31(6): 1087-1091 a b Fig. 4. 72-year old man with synchronous squamous cell and II 聊멘 c neuroendocrine carcinomas associated with idiopathic p 비 monary librosis a. CT scan with a mediastinal window setting shows a lobulating mass in right lower lobe(arrows) which was squamous cell carcinoma. b. CT scan at the lower level with a lung window setting shows honeycombing apperance c. Gross photograph 01 the cut surlace 01 the resected right lower lobe, which is identical to CT plane in (a), shows subpleural honeycombing(arrowheads) and mass(arrows) arising in it. The other mass, conlirmed as well differentiated neuroendocrine carcinoma, is not demonstrated in this photograph 는폐암은그원인에관계없이섬유화나만성염증에이은속발성상피세포의과형성의결과로알려져있다. 그리고미만성의간질성폐섬유화가증식하는상피의특이발암물질 (specific carcinogen) 에대한감수성에영향을미치는가능성도제기된바있다 (8, 9, 10). 섬유화성폐포염환자에서기관지폐포세척액 (bronch 경기관지생검에의하여폐암이진단된경우, 폐싫질에경결 (consolidation) 이나결절 (nodu le) 이있다하더라도바로그부위가폐암인지아니면다른부위에서생긴폐암인지정확한구분은불가능하다는점과, 폐섬유화증에통반된어떠한병변이있는경우감염에의한병변인지폐암에의한병변인지 CT소견만 로구별하기는힘들다는점이 oalveolar lavage f! uid) 에서의 암성태아성항원 / 알부민 다. (carcinoembryonic antigen / albumin) 의값은폐암이동반된경우높았다 (7). 이러한높은암성태아성항원의값은암전화생 (premalignant m e taplasia) 이나과형성 (hyperplasia) 의지표가될것이며폐암의위험도가높을것임을시사한다 (7). 본연구에서특발성폐섬유화증환자에서의폐암은섬유화가진행되는변연부에주로위치하였다. 이러한소견은폐암의발생이특발성폐섬유화증을포함한미만성폐섬유화증의섬유화와관련이있으며그종양은폐섬유화가일어난변연부에서자라서그 CT 양상이경계가불분명한경결양의종양으로나타난다고생각된다 (Fig. 1). 그러나본연구의문제점은폐암의진단방법상객담병리검사나 결론적으로특발성폐섬유화증환자에서폐암이동반되 는빈도는일반대중에서보다더높으며, 주로섬유화가진 행되는부위인변연부에서발생하였다. 이때 CT유형은경 계가명확하지않은경결영 : 의종양의행티l 로주로나타났 다. 따라서특발성폐섬유화증이있는환자의 CT 소견상 종괴양병변이발견된경우병발된폐암의가능성을항상 생각하여야한다. 7':~ C그 고 헌 1. Turne r-warwick M, Lebowitz M, Burrows B, Johnson A. Cryptogenic fibrosing alveolitis and lung cancer. Cancer 1980 1090 -
이학종외 특발성페섬유호흩에돔반된페암 ; 35 : 496-499 7. Fraser RG, Pare JAP, Pare PO, Fraser RS, Genereux GP. Di- 2. Haddad R, Massaro D. Idiopathic diffuse interstitial pulmon- agnosis of diseases of the chest. 3rd ed. Philadelphia ary librosis (tibrosing alveolitis), atypical epithel ial prol iler- Saunders, 1989: 1338-1339 ation and lung cancer AmJMed1968 ;45 :211-219 8. Fraire AE, Greenberg SO. Carcinoma and dilluse interstitial 3. Fraire AE, Greenberg SO, 0 Neal RM, Weg JG, Jenkins OE. librosis 01 lung. Cancer 1973 ; 21 :1078-86 Oiffuse interstitial librosis 01 the lung. Am J Clin Pathol 1973 9. Fox B, Risdon RA. Carcinoma 01 the lung and diffuse inter- ; 59 : 636-647 stitial p 비 monary librosis. J Clin Pathol 1968 ; 21 : 468-491 4. Watters LC, King TE, Schwarz MI, Waldron JA, Stanlord RE, 10. Spain OM. The association 01 terminal bronchiolar carcinoma Cherniack RM. A clinical, radiographic, and physiologic scor- with chronic interstitial inllammation and librosis 01 the ing system lor the longitudinal assement 01 patients with idio- lungs. Am Rev Tuberc 1957; 76: 559-567 pathic pulmonary librosis. Am Rev Respir Dis 1986 ; 133: 11. Meyer EC, Liebow AA. Relationship 01 interstitial pneumonia ; 97-103 honeycombing and atypical epithelial pr이 ileration to cancer 5. Scadding JG. Chronic diffuse interstitial librosis 01 the lungs. 01 the lung. Cancer 1965; 18 : 322-351 Br M J 1960 ;443-450 12. Stack BHR, Grant IWB, Irvine WJ, Moffat MAJ. Idiopathic dil- 6. Seo J-W, 1m J-G, Kim Y-W, Kim J-H, Sheppard MN. Synchron- luse interstitial lung disease. Am Rev Respir Dis 1965 ; 92 us double primary lung cancers 01 squamous and neuro- 939-48 endocrine type associated with cryptogenic librosing alveo- 13. Yokoo H, Suckow EE. Peripheral lung cancers anslng In I itis. T horax 1991 ; 46 : 857-858 scars. Cancer 1961 ; 14 : 1205-15 Journal of the Korean Radiological Society, 1994 : 31 (6) : 1087-1091 Lung Cancer in patients with Idiopathic Pulmonary Fibrosis: Frequency and CT Findings' Hak Jong Lee, M.D., Jung-Gi 1m, M.D., Joong Mo Ahn, M.D., Kyung Mo Yeon, M.D. 1 Department of Radiology, Seoul National University College of Medicine Purpose: The incidence 01 lung cancer in patients with idiopathic p 비 monary librosis(ipf) is higher than that 1 general pop비 ation. To evaluate the Irequency and CT lindings 01 lung cancer associated with idiopathic pulmonary librosis, we analyzed 19 patients with lung cancer associated with idiopathic p 비 monary librosis Materials and methods: We analyzed retrospectively 19 patients with histologically conlirmed lung cancer out 01 208 patients diagnosed as IPF either by CT and clinical lindings(n = 188) or histologically(n= 20). AII 19 patients were male, aged 40-85 years (mean 66 years). Scanning techniques were conventional CT in 12 patients, HRCT in 1 patient and both conventional CT and HRCT in 6 patients. We analyzed the CT patterns 01 lung cancer and IPF, locations 01 the tumor and histologic types 01 lung cancer Results: The incidence 01 lung cancer in patients with idiopathic p 비 monary librosis was 9.1 % (19 /208). In 11 01 19 patients, CT lindings 01 lung cancer were ill-delined consolidation-like mass. Lung cancer was located mainly in lower lobes(right lower lobe; 10/19, left lower lobe; 5/19) and at the periphery(12/19). Histologically, squamous cell carcinoma was the most common cell type (11 /19) Conclusion: The incidence 01 lung cancer in patients with idiopathic p비 monary librosis was much higher than that 01 general pop비 ation. Typical CT lindings 01 lung cancer were predominantly ill-delined consolidationlike mass at the peripheral lung portion which is the locatiaon where the most advanced librosis occur Index Words: Lung, librosis Lung neoplasms, CT Addr ess r eprint requests t o : H ak Jong Lee, M.D., D epar t ment of Radiology, Seoul National University College of M edicine # 28, Y on g on 겁 on g. Chongro-gu. Seoul, 110-744 K orea. Tel. 82-2- 760-2519 Fax. 82-2-743-6385 - 1091 -
1994 년도 50. 차학술대회증례퀴즈 ( 1 ) a b c Case 1. F/15 c.c. ;1) cough 2) blood tinged sputum D ; 1) 4 months 2)10days Dx ; pulmonary artery aneurysm & lung infarct in Behçet s disease a (93-5-4) b (93-7-5) Case 2. c.c. ;cough & blood tinged sputum D ; 3 months Dx ; Lumg torsion c (93-10-6) 1092