大韓放射線醫學會훌훌 Vol. XlX, No. 2, 1983 Castleman 病 ( 巨大 * 從隔洞微巴節增植 ) - 1[ 例報告 - 慶熙大學校훌훌科大學放射線科學敎室 成東릎 尹棒 成樂觀 崔祐碩 金聲鍵 - Abstract- Castleman s Disease (Giant Mediastinal Lymph Node Hyperplasia) Report of 2 cases Dong Wook 5ung, M.D., Yup Yoon, M.D., Nak Kwan 5ung, M.D., Woo 5uk Choi, M.D. and 500n Yong Kim, M.D. Deportmènt of Rodiology, Kyung Hee Unlverslty Hospitol Castleman s dîsease (Giant Iymph node hyperplasia) is a pathologic entity of unknown etiology, as attested to by the number of names it has received, Iymphoid hamartoma, angiomatous hamartoma, and giant Iymph node, etc. Althrough the mediastinum is its most common location, it also occurs in other areas of the body, usually where Iymph nodes are normally found. Authors have been experienced 2 cases of histologically proven Castleman s disease during recent 3 years in Kyung Hee University Hospital, and present its radiological and pathological findings as mediastinal mass. I. 績論 巨大 *1ft 隔洞뺨모節增뼈은 1954 年 Castleman 에의 해처음報告된이래 1 9 5 6 年 μ 後하나의病理學的흉 病으로確立되었다 1) 이흉思은被隔洞에가장好發 하나빼巴節이存在하는體 f1-3 의어느部 ÜI 에나發 ~ 할 : 느 1 rl 2) ~ -, 著者들은最近 3 年間慶熙大學校醫科大學附圍病院 에서談斷된故隔洞에생긴틴大없모節增꺼홉 報告하고아울러文없考察을하고자한마. II. i'leøtj 報告 이논문은 8 3 년 3 월 11 일에채택되였읍니다. 2 例를 :iæ"øjll 愚홈 ; 이 0, 27 歲, 男子 主訴및病歷 2 年빼부터間歐的인 I!!f<* 빛 左없 a ø행 部 * 홍痛이있었A며그당시뼈部 X 線上 前 *1ft 隔洞에 睡塊가發見되었으나그간漢方에서흉物服用을하여 오다가手術을위해本院에入院하였마. 過去歷상 J;ij 年 期때 IIIJ! 횟잦을앓은적이있었고 6 年前結核으로응? 斷 하여 1 年間藥物服用하였으나, 이 당시睡塊의存 te 有 無는확실히알수없었마. 理學的所見 : 正常 病理檢훌所見 : 入院당시血被所見은血色素 11. 9 밍n %, S 血球 8, 200-{ rnrn 3, ii}; 血球容積 39 % 이었고l 두번에결친嘴짧뽑養檢홉에서약간의 ps eud om onas와 알파連銷狀球園이자라는것이외에略찢의結核홈養및 - 367-
용妹檢좁에서는陰性이었마 口가약간俠염되고充血되어있었다 斷 I 험짧影術上左 放射線學的所見 : 單純뼈部擬影에서 (Fig.l) 左뼈 IJ 上部師에 4 x3 cm 의睡塊가存在하였으며左上部心職緣이消失되었다. 예뾰擬影에서睡짧는中般隔 /fpl 에뾰뽑한것을알수있었마. 思者는氣管支鐘檢훌를施行하여氣管分支部의角度 (carinal 없gle) 가날카롭지못하 上葉氣管交에약간의썼캘이있을뿐, 탬때內에는石 Ð< 化沈월은없있다- 大動服 IfIl~ 풍造影術上陣뼈는 lftij IDR 德가이념을일수있었다 (F ig. 2). 入院 20 日째閒放性開뼈術을 lj' 施하였다手術所見 : 暗 ~q용色의주먹크기의睡!\15가左上뚫의 었 며, 右氣管支는正常인데반해左上葉의 上分節入 師門가까이에위치하였으며이것은上핏까지 침범하였 Fig. 1. PA view of the chest shows a soft tissue mass in the left upper chest with obliteration of left upper cardiac border. In lateral view, visible mass is located in middle mediastinum. Fig. 2. Tomogram, bronchogram and aortic angiogram reveal slight stenosis of superior segment of left upper lobe. And the mass is not communicated with aorta. -3 68 -
고 6 ~7 개의多發性結節性睡塊로만져졌다. 病理組織學的所見. 肉眼的으로左뼈門部位에몇개 의비후된結節性組織이存在하였우며, 이중가장큰 것이 4 x 3 x 2.5 cm 되었다. 績斷面은出血을 동반한 Ek S 色의均質의肉塊樣相을보였다 顯微鏡的所見은 빼밍組織의 i않度增꺼흡이 散在하였으 며, 시 8영間中心細 8영 ( f이 li c ul a r-c ent e r c e lls ) 들이 Ha s sall 小짧 (Hassal! s corpuscle) 와비슷한구조를지 녔으며周圍의뼈子質에의해 同心性 (concentric ).!?. 로 관찰되었다 (F ig.3. A). 그러냐 Hassall 小體에서 보이는 kera tin 은存在하지않았 며小 8 包間 部 ill에는 뼈子質化된毛細血管增뼈이特徵的이었.!?. 며 形質細 8힘 (plasma cell ) 가好짧球 (eosinophi 1) 와더붙어섞여있 <, 1 1,3> λλ-, :iæ'ølj 2 똘 者 : 조 o 0, 1 7 歲, 男子主訴및病歷 3 年前부터빈벤한감기효狀과 @ 홈 ~ 이있었으며 { 固 A 짧院에서 n행즙ß X 線題影 J: ol] 서짧隔洞에障짧가發見되어本院에入院하였다過去歷에 13 才때結核으로인해 2 年間抗結核剛치료플받은적이있었마. 또한아버지와형제들에게結核의 i댐去짧이있었다. 理學的所見 : 正常 타所見및略짧檢좁모두陰性이었다. 放射線學的所見 : 單純뼈部提影 t에서軟組織睡!m 陰影이大항b Hm 련파뼈骨사이의 ffii *iæ 隔洞에 f 立置하였으며 mm{ 七斷 l협짧影術 ( C T) 를寶施하여 ( F ig.4) 大動服련의前삐 { 立폐애點狀의石 Ek 化沈휩을지난前 *iæ 隔洞 R훨傷으로등? 斷하고入院 5 日째開放性開 O행術 (open thoracotomy ) 를實施하였다 手術所見 : 어린이주먹크기의탄력성이있는睡塊가前없隔洞에位置하였으며그外面은뼈服組織과정해있었고나머지部位는被뼈 ( encapsula t i o n) 되어쉽게박리가되었다. 病理組織學的所見 : 重풀 100 gm, 5.5 x 4.5 cm의크기를지니며짧斷面은軟黃色의均質한肉!m t옳相을이루고있었으며 n때服에서볼수있는繼維桂 (Trabecul - a tion ) 은볼수없었마. 顯微鏡的所見은血管 t흡짧이顯著하였고, 주변부에 1iX. 熟한빼巴球가 I협을이루고있 3며뼈子質化펀없巴모양의小 8 包들이中央에 f 立置하여마치양파껍질모양 (oni on- skin appea r a n ce) 을나타내었다 (Fig.3 B ) 10>. 일부外面에접해있는뼈願組織에는벨 l 상이없었다. ßI. 考 按 病理倫훌所見入院당시 m 硬所見은 rfrr 色뚫 11.8gm 敬隔洞에생긴巨大財巴節增植은아직확실히그 %, S 血球 5900 / mm 3,!iF 血球容趙 37 % 이었고, 기原因을알수없으냐 # 巴節의非特暴性慢性갖 tle 圖程에 A B Fig. 3. (A) Diffuse hyperplasia of lymphoid follicle, surrounded by concentric layers of lymphocytes. Interfollicular portions show prominent proliferation of capillary structures. These structures may be mistaken for Hassall s corpuscles. Hematoxylin and Eosin; x40 (B) The lesion is characterized by prominent vascular proliferation and hyalinized center of the lymphoid follicles with layering of the mature lymphocytes at the periphery, resulting in an onion-skin appearance. Hematoxin and Eosin; xloo - 369-
Fig. 4. Chest P A view revea1s soft tissíle mass density in the mediastinum latera1 to aortic arch. Computed tomography of chest show a mass density with some punctate calcifications in the left anteriro mediastinum between sternum and aortic arch. 의한다는說이지배적이다 1, 4, 5,6) 황j 離하지옷했을때는再發할수가있다 12) 또한過 Ke l1er 等 2) 에의하면이흉愚은빼巴節이存在하는度血管增碩으로인해手術시出血이적지않다는것을體內의어느部뾰에나發生할수있 나敬隔洞에가장염두에두어야한다 ::S, 1ι J 手術前의뼈血管性病뚫와好發한다고하였고, Latters 좋7l은없모節에서생겨나감별하기위해血賢振影術이나放射性同位元素走훌法지않은 m E'3 節모양의睡塊 1 2 例를報告함으로써야 * 이鍵別談斷에도웅이되며, 이때의所見은後期 ( later 모節이없는體內어느部뾰에서도생겨날수있다고하 stage) 에過度血管增題樣相을보이게된다 14,15) 였다. Ke l1er 等 2) 은病理組織學的 o 로 cas tleman 病을뼈子質血賢性型 (hyaline-vascular type) 과形質細 8힘型 (plasma cel1 type J ~ 로區分하고, 홈6 者 (9 1 % ) 는작은碩子質血管性小 8명 (hyal ine~va s cul ar f 011 icle ) 와小 8힘間毛細血管 t 曾꺼홉이特徵的이며主로單一圓形睡塊를形成하며 l 形質細뼈型은비교적큰小 8힘들과더불어形質細뼈의 I험이小뼈間組織에存在하며多發性인分離된빼밍節들이睡塊플形成하는것이特徵的이다 5, 8, 9,10) 報告된대부분의例에서이용愚으로인한!lE 勢는없었으며우연한기회에 K뚫塊를發見하게되었다 9, 10, 11, 1 야形質細뼈型에서는가끔홉血, hypoglobulinemia, 發熱, 發규들의숲身효勢가나타날수도있다 3,12,13) 초로젊고건강한사람에게장생기며 70 % 가 30 세以下의 나이에好發한다고報告하였고男女의性別차이는 없 다고報告되었으나 4, 8, 1 2, 1 3) 著者들은男子 2 例룰경험 하였마. 治續로는기능한 B웹手術하는것이좋으며운숲히제 거시는再發이나陣移 (metastasis) 하지않으나完숲히 著者들은最近 N. 結論 3 年間慶熙大學校醫科大學附團病院 에서경험한敏陽洞睡짧의所見을보인 Cast eman 病 2 例를經驗하였기에文敵考察과더불어報告하는바 이마. REFERENCE 1. Castleman B, Iverson L, Menendex V Local/zed mediastlnal Iymph node thymoma. Cancer 9:822.830, 1956 hyperplasla resemb l/ng 2. Keller AR, Hochholzer L, Castleman B Hyal/nevascular and plasmacel/ hyperplasla of the medlastinum and other locatlons. Cancer 29:670-683, 1972 3. Walter J F, Rottenberg R W, Cannon WB et al Giant medlastlnal Iymph node hyperplasla: Anglographlc and clinlcal features. Aj R 130:447450, 1978-370 -
4. Bloch C, Peck HM Radiological notes. Case No. 778. Locallzed benign Iymph node hyperplasia, rlght h/lum (Castleman 's dlsease). j Mt Slnal Hosp 29:364-366, 7962 5. Wolfel DA, Antorijus )1, Cowley RA posterlor medlast!nallymph node hyperplasla. AjR 97 : 720-724, 7964 6. Horie A On the so-called lymph node hyperplaslu" (castlemon). Kyushu j Med Sci 73: 705-770, 7962 7. LattersR, Pachter M Benlgn Iymphold mosses of prqboble homortous noture: Cancer 75:797-274, 7962 Anolysls of 72 coses. 8. Griff LC, Griff RE Mediastinal Iymph node hyperplosla. BjR 40: 68-69, 7968 9. Inada K, Kawai K, Katsumura T et al Glant /ymph node hyperplosio of the medlostinum. Am Rev Tuberc 79:232-237, 7959 10. Tung KSK, McCormack 니 Angiomatous Iymphoid hamortomι Concer 20:525-536, 7967 11. Katz 1, Dziadiw R Locolized medlostlnol Iymph node hyperplosia. AjR 84:206-272, 7960 12. Olscamp G, Weisbros G, Sanders D et al Castleman dlseose: Unusual monifestatlons of an unusual disorder. Radiology 735:43-48, 7980 13. Harrison EG, Bernatz PE Angiofol!icular mediostinal Iymph node hyperplaslo resembling thymoma. Arch Pathol 75:284-292, 7963 14. Funamura )L, Uszler )M, Torrance D) Benlgn ongiofol!icular /ymph node hyperplasia-demonstration of systemic arterial perfusion by dynamlc lung circulation scinelgrnphy. j Nuc/ Med 27 :600-607, 7980 15. Tuttle RJ, Shier K) Angiography of angiomatous Iymphoid hamartoma and 0 suggested pothogenesls. Rodiology 730:377-375, 7979 16. Bersack 5 R, Howe )5 Asymptomatic mediastlnal Iymph node hyperplasio of eighteen years durotion. Dis Chest 43:443-446, 7963-371-