대한망사선의학회지 1997; 37: 악성후복막강섬유증의방사선학적소견 장유진 이혜경 김형환 차장규 홍현숙 권귀향 최득련 목적. 악성후복막강섬유증의방사션학적소견을알아보고자하였다. 대상및방법 : 악성후복막강섬유증으로진단된 9명의 CT 및요로조영술소견을후향적으로

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1 대한망사선의학회지 1997; 37: 악성후복막강섬유증의방사선학적소견 장유진 이혜경 김형환 차장규 홍현숙 권귀향 최득련 목적. 악성후복막강섬유증의방사션학적소견을알아보고자하였다. 대상및방법 : 악성후복막강섬유증으로진단된 9명의 CT 및요로조영술소견을후향적으로분석하였다. 모든환자에서조영증강후 CT 사진을얻었고, 이중 7명의환자에서요로조영술을얻었다. 대상의원발성암으로는진행성위암 (n=6), 조기위암 (n=l), 유방암 (n=l), 자궁경부암 ( n= l) 이었다.CT상연조직병변의위치, 요관침범여부, 수신증의유무, 다른장기로의전이소견등을분석하였고요로조영술상요관의침범부위, 침범길이, 요관협착유무빛전위등을분석하였다. 결과 :CT상전예에서고밀도로조영증강된연조직병변 ( 연조직종괴 :5 예, 연조직판 :4예) 이복부대동맥및하대정맥주위에서관찰되었고그중 8예에서는요관벽의비후가동반되었다. 수신증은전예에서있었고이중 2예는양측성이었고 7예는편측성이었다. 럼프절비대는 5예에서관찰되었다. 요로조영술상 7예모두에서다양한부위 (L2-S2) 의불규칙한요관협착과요관의내측전위가관찰되었고침범된요관의길이는 4-6.5cm이였다. 결 론 : 본연구에서악성후복막강섬유증의흔한 CT 소견은복부대동맥빛하대정맥 주위에조영증강되는연조직병변과수신증및요관벽의비후였다. 또한요로조영술에서 는요관협착과요관의내측전위가관찰되었다. 후복막강섬유증은제 4 혹은제 5 요추위치에있는장골혈관 ( 복부대동맥분지 ) 주위에섬유판 (fibrou s plaque) 이형성되고다 % 댄정도의섬유증식이후복막강에생기는질환으로편측, %t측, 혹은국한된 ( circumscribed) 형태로나타나는비교적드문질환이다 (1, 2). 이중약 70 % 가특발성이고나머지가이차적인원인 ( 약물, 대동맥류 ) 혹은악성종양등여러가지원인에의해초래된다고보고되고있다 (3-5 ). 저자들은특발성후복막강섬유증과는다른악성후복막강섬유증에서 CT 와요로조영술소견을분석하여악성후복막강섬유종으로진단하는데도움이되는소견을알아보고자하였다. 성후복막강섬유증으로진단되었고나머지 4예는조직학적으로증명되지는않았으나과거력상원발성암이있어악성후복막강섬유증으로진단이가농하였다. 대상환자중남자가 5명, 여자가 4명이었고진단당시연령은 25세부터 69세로평균연령은 47세였다. 9명모두에서 CT 촬영을조영제 Iopamiro 370(0.752g /1ml of iopamidol, Ilsung, Korea) loom l을급속히정맥주사한후시행했으며, HITACHI CT-W2000(Hitachi Medical Corp., Tokyo, Japan) 기종을사용하여간의상부에서부터방광의기저부까지절편두께 lomm, 간격 l Omm로스캔하였다. 이중 7 명에서는경정맥 (4예) 혹은역행성신우요관조영술 (5예) 이 시행되었다. 대상및방법 년 12 월부터 1996 년 7 월까지원발성암의병력이있거나 원발성암과동반되어복부둔통혹은수신층을주소로내원한 9 명의환자를대상으로 CT 와요로조영술을후향적으로분석 하였다. 이들중 5 예는수숨을시행하여병리조직학적으로악 l 순천향대학교의과대학진단방사선과학교실 이논운은 년 1 월 28 일접수하여 1997 년 8 월 11 일에채택되었음 각각의환자에서 CT상보이는연조직병변의위치, 요관침범여부, 수신증의유무, 다른장기로의전이소견등을분석하였다. 함께시행한요로조영술이있을경우는요관을요관신우접합부에서요관방광접합부까지장골혈관분지 (bifurcation of iliac vessels) 를기준으로복부와골반요관으로나누었다. 다시복부요관 (abdominal ureter) 을이등분하여상부, 중부요관으로나누었고골반요관을하부요관으로구분하였다. 요로조영술상요관의침범부위, 침범길이, 협착유무및전위

2 장유진오 1: 악성후복막강섬유증의밤시선학적소견 등을분석하였다. 침범된요관분절의길이는 4-6.5cm이었다 (Fig. 2A, 3A). 7~ , 고 찰 각환자에대한자세한방사선학적소견은 Table 1 과같다. 원발성종양으로는위암이 7 명으로가장많았고, 유방암이 1 명, 자궁경부암이 1 명이었다. 6 례의진행성위암환자는수술 당시뱅기 m 이상이었고, 그중 3 예는원격전이 (distant metastasis) 가있었다. 유방암은침윤성선암종 ( infiltrating ductal carcinoma) 으로병기는 T2 N 1 M 이었다. 원발성암이진단되고악성후복막강섬유증이발견될때까 지의 7 1 간은 3개월에서 53개월로평균 24개월이었다. 동반된 수신증이발견될당시의증상은상복부불쾌감 3 명, 복부둔통 4 명, 암구리통증 2 명이었으며대부분급성증상으로시작되였 다. 총 9 명의 CT 상광범위한후복막강내연조직종괴을보인예 가 5 예 (Fig. la, 3B, C) 였고, 연조직판은 4 예 (Fig. 2B, C) 에서 보였다위치는상부요관에서하부요관까지다양한요관분절 을침범하였다연조직음영은조영증강후에는전예에서요근 에비해모두고음영으로보였고주변혈관과의경계가불분명 하였으나, 주변혈관의위치전위는보이지않았다 (Fig. C). CT 상다른소견으로는수신증이전예에서보였고 2 예에서 lb, 는 o.}: 측요관을 7 예에서는편측요관을침범하였다 ( 우측 :3 예, 좌측 :4 예 ). 또한요관벽의비후 ( thickeni ng) 를 8 예에서보였 다 (Fi g. 2B, C). 악성후복막강섬유증과동반된복강내이상 소견으로는복수가 3 명에서있었으며, 비대는 5 명 (5/9) 에서있었다. lcm 이상크기의럼프절 요로조영술을시행한 7 예에서다양한요관부위 (L2-S2) 에 서불규칙적인요관협착과요관의내측전위가관찰되었으며, 후복막강섬유증은섬유질의증식이대개하부요추및천골갑각 (promontory) 에서시작하여요관을따라양측으로진행되어제 2 혹은제 3 요추부위까지침범하는질환으로알려져있다. 이질환은약 70% 가량이특발생으로초래되고, 나머지가대동맥류, 약물남용 ( 특히 methylser -gide, metherogotamine, methyldopa), 종양, 염증에의해서이차적으로발병한다. 복부대동맥류에의한동맥류주위섬유층이이차적원인으로는가장흔한것으로알려져있다 (6-8). 특발성후복막강섬유증으로온경우에는남자에서여자보다 2-3배번번히발생하며호발연령은 세로되어있다 (3). 조직학적으로특발성후복막강섬유증은다양한정도의섬유증 (fibrosis ), 유리질화 (hyalinization), 지방육아종증 (lipogranulomatosis), 그리고염증성단핵세포의섬유교원질 (fibrocollagenous stroma) 로구성된다 (9). 또한염증과세포반응 ~ 은주로초기병변에서나타나며만성병변에서는감소된다. 반면악성후복막강섬유증은악성세포가후복막에침윤 (infiltration) 도1 7-] 나결합조직형성과경화성의반응 (desmo plastic and sclerotic reaction) 을일으켜발생한다 (1 0, 11). 임상증상은비특이적이며복부및옆구리동통, 체중감소, 위장관불쾌감등을호소한다. 경한수신증에비하여진단받을당시약 40% 의환자에서신부전이발견되며수신증은요관의연동운동방해와압박에의하여생긴다. 악성후복막강섬유증진단에병력이도움이될수있으며예후는일반적으로매우나빠서진단후생존기간이 3-6개월정도로알려져있다 (11). A B Fig. 1. A 56-year-old man who was diagnosed as advanced gastric cancer 10 months ago (Case 1) A, B. Abdominal CT scans show ill defined enhancing soft tissue mass(arrowheads) in prevascular area and multiple fat strands in left paraaortic area. Dilatation of left ureter(small arrowheads)is also noted. 一 900 -

3 No. --대한방시선의학회지 1997: 37: A Fig. 2. A 30-01d woman with gatrectomy for advanced gastric cancer (Case 2). A. A retrograde pyelography shows hydronephrosis of the left kidney. The left ureter shows segmental stenosis and medial displacement at the level from L3 to L4 (arrowheads) B, C. CT scans show irregular enhancing soft tissue mass (arrowheads) in IVC and left paraaortic area with left hydronephrosis. Circumferential thickening of left ureteral wall is also noted(arrow). c Table 1. Summary of Cases of Malignant Reπoperitoneal Fibrosis Case 이ι1j A4 R J ro / n? Sex/Age M/s 6 F/30 M/64 F/68 M/69 M/25 M/63 F/64 F/59 Primary Tumor Site EGC Breast Cervix Note --RPF: retroperiotneal fibrosis Shape of RPF Plaque Mass H ydronephrosis Rt Rt Both Both Rt Level : the level of ureteral involvement RGP: retrograde pyelography EGC : early gastric cancer IVP : intravenous pyelography : advanced gastric cancer Rt / : right/ieft M: male F : female Thickining of U reteral Wall RGP L3 - L4 L5 - S2 L3 - L5 L2 - L4 L4 - L5 Level* IVP L5 S2 L5 Sl L3 - L5 L4 - L5-901

4 장유진오 1: 악성후복막강섬유증의망시선학적소견 Fig. 3. A 64-01d woman who was diagnosed as breast cancer 4 years ago (Case 8). A. A retrograde pyelography shows irregular narrowing of left proximal ureter and diffuse stenosis with medial displacement of left mid-ureter at the level from L2 to L4 (arrowheads). B, C. CT scans show ill defined enhancing soft tissue mass(arrowheads) enveloping the aorta, IVC, and both ureters and bilateral hydronephrosis. C 악성종양에의해초래되는경우대개 3 가지원인으로구별 될수있다. 이원인으로는 1) 요관주위로의전이암 2) 원발성 후복믹종양 3) 칼시노이드종양에의한세로토닌분비등이다 (12, 13). 요관을따라전이를잘하는원발암으로는유방, 위, 전립선, 폐, 콩팔, 대장등이다 (1 4). 이때에는요관주위의섬유지방조직과럼프관에국한하여 섬유증식되어요관을둘러싸거나, 골반, 복강및후복강내로 광범위하게퍼진암종증의일부로나타나요관주위조직을따 라침윤및 결합조직을형성하여요관의 폐쇄를초래한다 (15-17). 후복막강섬유증의고식척인방사선학적진단방볍으로는단 순촬영 (KUB), 경정맥빛역행성신우요관조영술등이이용 된다. 역행성선우요관조영술상요관의불규칙한협착및폐쇄 소견을보이면서하부요추부위즉요관하부부위에서요관의 내측전위를보이고상부수뇨관및요관의확장이전형적인소 견이다 (6). 그러나요관의내측전위는약 6 % 의정상인에서도 관찰되는것으로되어있다 (1 8). 요관폐쇄에의한수신증은초음파상쉽게발견되며비만흑 은장내가스때문에수반되는연조직의섬유화변화는거의알 기어려운것으로알려져있다 (1 9, 20). 후복막강섬유증의 CT 소견은후복막강의복부대동맥및 하대정맥주위에조영증강되는연조직병변과상부요관의확 장소견으로특정지워진다고한다 (2 1). 섬유증병변은복부대 동맥, 하대정맥앞에위치하며그들혈관의전 측연을강싸나 대개완전히에워싸지는않는다. 또한병변은측면으로진행되 어요근과의경계가불분명해지고요관을내후면부터둘러싸 전체를감싸나대개전외측부위는침범하지않는다. 복부대동맥혹은하대정맥등대혈관의전방전위는특발성 후복막강섬유증에서는입증되지않은소견으로악성후복막강 섬유증에서특징적으로일어나는것으로알려져있다 (6). 저자 들은 CT 상나타나는병변의위치, 혹은조영증강되는형태로 발병원인에대해감별하려하였으나신뢰할만하지못하였다. 그러나 CT 는후복막강섬유증의정도와인접후복막강내의 구조물 ( 대혈관, 요관등 ) 침범여부등을수술전에비교적정확 히알수있다. 최근자기공명영상에서비악성후복막강섬유증은항상명확 하게경계가그려지나, 악성후복막강섬유증은불분명한경계 를보이고또한 T2 강조영상에서인접요근에비해고신호강도

5 대한발사선의학회지 1997; 37: 와비균질성이자주관찰된다고보고되고있다. 즉자기공명영 상은 CT 와는달리형태학적특정보다는조직대조도에차이가 있음을이용하여감별할수있다고한다 (22, 23). 결론적으로본연구에서악성후복막강섬유증의원인질병 으로는진행성위암이압도적으로많았으며, 악성후복막강섬 유증의흔한 CT 소견은복부대동맥및하대정맥주위에조영 증강되는연조직병변과요관벽의비후및수신증이었다. 요로 조영술에서는요관협착과요관의내측전위가관찰되었다. 이 러한악성후복막강섬유증의 CT 소견은이미보고된바있는 특발성후복막강섬유증의 CT 소견과감별되지않았으며, 이 런경우임상병력, 자기공명영상이도움이되리라생각된다. 참고문헌 1. Ormond JK. BilatearI ureteral obstruction due to envelopment and compression by an inflammatory retroperitoneal process. J Uro/ 1948 ; Dalla-Palma L, Rocca-Rossetti S, Pozzi-Mucelli RS, Rizzatto G Computed tomography in the diagnosis of retroperitoneal fi brosis. Urol Radio/ 1981 ; 3 : Lepor H, Walsh PC. Idiopathic retroperitoneal fibrosis. J Uro/ 1979; Koep L, Zuidema, GD. The clinical significance of retroperitoneal fi brosis. Surgeη 1977; Ormond J K. Idiopathic retroperitoneal fibrosis: A discussion of the etiology. J Uro/ 1965; 94: Degesys GE, Dunnick NR, Silverman PM, Cohan RH. IIIescas FF, Castagno A. Retroperitoneal fibrosis: use of CT in distinguishing among possible causes. AJR 1986 ; 146: Brun B, Laursen K, Soerensen IN, Lorentzen JE, Kristensen JK CT in retroperitoneal fibrosis. AJR 1981 ; 137: Dixon, AK, Mitchinson MJ, Sherwood T. Computed tomographic observations in periaortitis. C/in Radio/ 1984; Osborne BM, Butler JJ, Bloustein P, Sumner G. Idiopathic retroperitoneal fibrosis (sc!erosing retroperitonitis). Hum Patho/ 1987; 18: Usher SM, Brendler H, Ciavarra V A. Retroperitoneal fibrosis secondary to metastatic neoplasm. Ur%gy 1977; 9: Grabstald H, Kaufman R. Hydronephrosis secondary to ureteral obstruction by metastatic breast cancer. J Uro/ 1969; 102: Mieza M, Rotstein J,Geffen A. CT demonstration of periureteral fibrosis of malignant etiology. J Comput Assist Tomogr 1982; Ambos MA, Bosniak MA, Megibow AJ, Raghavendra B Ureteral involvement by metastatic disease. Uro/ Radio/ 1979; 1 : Winalski CS, Lipman JC Tumeb SS. Ureteral neoplasms RadioGraphics 1990; 10: Bosniak, M., A. Megibow, M. Ambos, J. Mitnick, R. Lefleur, R. Gordon Computed tomography of ureteral obstruction. AJR 1982 ; Megibow, A., J. Mitnick, M. Bosniak. The contribution of computed tomogra phy to the evaluation of the obstructed ureter. Uro/ Radio/ 1982; (4) 이재영, 검숭협, 컴태성등전이성요관암의 CT 소견 - 대한방사 선의학회지 1995; 33(5): Saxton HM, Kilpatrick FR, Kinder CH. Lessof MH. Mchard y-young S, Wardle DFH. Retroperitoneal fibrosis: a radiological and f, 이low-up study of fourteen cases. Q J Med 1969 ; 38: Fagen CJ, Larrieu AJ, Amparo EG. Retroperitoneal fibrosis: Ultrasound and CT features. AJR 1979 ; 133: Feinstein RS, Gatewood OMB, Goldman SM, et al Computerized tomography in the diagnosis of retroperitoneal fi brosis. J Uro/ 1981; 126: Brun B, Laurser K. Sorensen IN, Lorentzen JE, Kristensen JK CT in retroperitoneal fibrosis. AJR 1981 ; 137: Arrive L. Hric 따 H. Tavares NJ, Miller TR. Malignant versus nonmalignant retroperitoneal fibrosis : differentiation with MR imaging. Radi%gy 1989; 1 72: Marineck B, Scheidegger JR, Studer UE, Kraft R. Metastatic disease of the ureter: patterns of tumoral spread and radiologic fidings. Abdom Imaging 1993; 18: Kerr, W.S., Jr., Suby, H.I., Vickery, A. and Fraley, E. Idi pathic retroperitoneal fibrosis: clinical experiences with 15 cases. J Uro/ 1968; 99 :

6 장유진오 1: 악성후복막강섬유증의밤시선학적소견 J Korean Radi 이 Soc 1997; 37: Radiologic Findings of Malignant Retroperitoneal Fibrosis Yu Jin Chang, M.D., Hae Kyung Lee, M.D., Hyung Hwan Kim, M.D., Jang Gyu Cha, M.D., Hyun Sook Hong, M.D., Gui Hyang Kwon, M.D., Deuk Lin Choi,M.D. 1 Department of Radiology, College of Medicine, Soonchunhyang Universiη Purpose: To evaluate the radioloic findings of malignant retroperitoneal fibrosis Materials and Methods: Post-contrast CT (n=9) and urographic (n=7) findings of nine patients with malignant retroperitoneal fibrosis were retrospectively analyzed. Primary tumors were found to be advanced gastric cancer (n=6), early gastric cancer (n=l), breast cancer (n=l), and cervical cancer (n=l) We analyzed CT findings with regard to the site of soft tissue lesion, ureteral involvement, the presence or absence of hydronephrosis, and distant metastasis. The level and length of ureteral involvement, presence or abscence of ureteral stenosis, and ureteral displacement as seen on urography, were analyzed. Results: On CT scans, enhanced soft tissue lesions (mass, 5 cases; plaque, 4 cases) encircling the abdominal aorta and IVC were noted in all cases. Thickening of the ureteral wall (n=8), hydronephrosis (n=9), and enlarged lymph node (n=5) were also seen. On urography, irregular stenosis and medial displacement of ureters from level L2 to S2 were noted in a11 cases. The length of ureteral involvement was 4-6.5cm. Conclusion : The common CT findings of malignant retroperitoneal fibrosis were enhanced soft tissue lesion encircling the abdominal aorta and IVC, hydronephrosis, and thickening of the ureteral wa11. On urography, ureteral stenosis and medial displacement were seen. Index Words: Retroperitoneal, space, CT Retroperitoneal, space, fibrosis Retroperitoneal, space, MR Retroperitoneal, space, neoplasms Address reprint requests to: Yu Jin Chang, M.D., Department of Radiology, College of Medicine Soonchunhyang University Hospital, # Hannam-Dong Yongsan-Gu, Seoul, Korea. Tel Fax

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