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1 대한내과학회지 : 제 76 권부록 1 호 2009 비장농양으로보인원발성비장림프종 1 예 인제대학교의과대학부산백병원내과학교실 최바울 박상원 강명주 정은욱박은택 이상혁 설상영 case of primary splenic lymphoma presenting as a splenic abscess Paul Choi, M.D., Sang Won Park, M.D., Myung Ju Kang, M.D., Eun Uk Jung, M.D., Eun Taek Park, M.D., Sang Hyuk Lee, M.D. and Sang Yong Seol, M.D. Department of Internal Medicine, Inje University College of Medicine, usan Paik Hospital, usan, Korea Primary splenic lymphoma (PSL) is very rare, with a reported incidence of less than 1% of all malignant lymphomas. The differential diagnosis of a solitary splenic mass should include benign entities, such as hemangioma, hamartoma, simple cyst, and metastatic carcinoma, as well as abscess. We report a case of primary splenic lymphoma presenting as a splenic abscess. 27-year-old man presented with left upper quadrant pain for 3 days. bdominal computed tomography (CT) revealed a splenic mass, a well-defined hypodense lesion (4.5 4 cm) in the posterosuperomedial aspect of the spleen. The patient improved clinically with percutaneous drainage of the spleen cystic mass and antibiotic therapy. fter 8 months, he presented again with left upper quadrant pain and fever. bdominal CT revealed progression of the splenic mass (5 8.5 cm) with some internal hemorrhage. The patient underwent splenectomy for a definitive diagnosis and treatment. t surgery, a splenic tumor was found with no evidence of it involving other sites, and it was confirmed pathologically as a diffuse large cell lymphoma of the spleen. (Korean J Med 76: S49-S53, 2009) Key Words: Primary splenic lymphoma; Splenic abscess 서론비장의원발성림프종 (primary splenic lymphoma, PSL) 은그발병률이모든림프종환자의 1% 미만을차지하는매우드문질환이다 1). 비장의원발성림프종의정의에대해서는아직논란이있으나, 일반적으로말초림프절종대나간및 복강내림프종의병변없이림프종이비장과비문림프절에국한된경우를말한다 2,3). 원발성비장림프종의진단은임상적증상외에복부초음파검사나복부전산화단층촬영 (computed tomography, CT) 등의방사선소견이도움이되는데대세포형림프종의경우저음영의비장종괴로나타나는경우가많아비장의농양, 단순낭포, 혈관종 (hema- Received: ccepted: Correspondence to Eun Taek Park, M.D., Division of Gastroenterology, Department of Internal Medicine, Inje University College of Medicine, usan Paik Hospital, Gaegeum-dong, usanjin-gu, usan , Korea euntpark@hanmail.net - S 49 -

- The Korean Journal of Medicine: Vol. 76, Suppl. 1, 2009 - Figure 1. () Doppler showed a voluminous spleen and a well-marginated 4.

5 4-cm hypodense lesion in the spleen containing hyperdense foci and a moderate fatty liver. ngioma), 전이성악성종양, 과오종 (hamartoma) 등과의감별진단이어렵다.

2 - The Korean Journal of Medicine: Vol. 76, Suppl. 1, Figure 1. () Doppler showed a voluminous spleen and a well-marginated cm low-echoic mass in the superomedial aspect without visible intramural vascularity. () On the first admission, abdominal CT indicated a well-defined cm hypodense lesion in the spleen containing hyperdense foci and a moderate fatty liver. ngioma), 전이성악성종양, 과오종 (hamartoma) 등과의감별진단이어렵다. 저자들은좌상복부통증을주소로내원후비장농양진단하에비장농양경피적배액술 (percutaneous drainage) 및항생제요법으로임상증상이호전되었던환자가 8개월뒤복통및방사선소견이악화되어비장절제술을시행한결과, 수술소견및조직검사에서원발성비장림프종으로확진한환자를경험하였기에보고하는바이다. 증례환자 : 하 안, 27세남자주소 : 좌상복부통증현병력 : 본원내원 1개월전좌상복부통증으로인근병원방문하여비장농양으로진단하에입원하여항생제치료받고증상호전되어퇴원하였으며, 내원 3일전부터좌상복부통증이다시발생하여본원으로전원되었다. 과거력, 가족력 : 특이소견없음. 이학적소견 : 내원당시의식은명료하였으며, 혈압 140/ 80 mmhg, 심박수 60회 / 분, 체온 36.7, 호흡수 22회 / 분이었다. 복부촉진상좌상복부통증이있었고, 진찰소견에서다른특이사항은없었다. 검사실소견 : 일반혈액검사에서백혈구 10,210/mL, 혈색소 14.4 g/dl, 혈소판 319,000/mm 3, 적혈구침감속도 (erythrocyte sedimentation rate, ESR) 13 mm이었다. 종양표지자검사에서 FP <0.61, CE 1.01, C 이었다. 생화학적검사에서 Total protein 7.4 g/dl, lbumin 4.2 g/dl, Total bilirubin 0.5 mg/dl, ST 26 U/L, LT 35 U/L, LP 161 U/L, rgtp 35 U/L, LDH 986 U/L이었다. PT/PTT 13.9/ 35.9 sec (INR 1.02), UN/Cr 11/0.9 mg/dl, CRP 1.08 mg/dl, mylase 59 Unit이었으며소변검사는정상이었다. 복부초음파및전산화단층촬영소견 : 복부초음파에서비장비대소견과함께비장의상부내측부위에경계가명확한 cm의저에코의 (hypoechoic) 종괴소견보였으며벽내의혈관분포 (intramural vascularity) 는보이지않았다 ( 그림 1). 복부 CT에서는비장비대소견과함께비장의후상부내측부위에안쪽으로약간의고음영의병소가동반된 cm 크기의경계가명확한저음영의낭종 (cystic mass) 소견을보였으며중등도의지방간소견이동반되어있었다 ( 그림 1). 임상경과및치료 : 내원당시복부 CT에서 cm 크기의경계가명확한저음영의낭종소견을보여비장농양의진단하에비장농양경피적배액술과항생제요법을시행하였다 ( 그림 2). 소량의농양이배액되었고, 배액세포검사 (cytology) 에서악성세포는관찰되지않았다. 경피적배액술 7일째시행한복부 CT ( 그림 2) 에서이전과차이가없어재발및악성종양가능성때문에조직검사및수술을권했지만, 통증소실등의임상증상의호전되자환자는수술을거부하고비장도관을제거후퇴원하였다. 이후 1개월뒤외래에서촬영한복부 CT상에서이전과차이가없었다. 이후 8개월뒤좌상복부동통및발열소견으로다시내원하여시행한복부 CT에서 cm 크기의저음영의낭종및낭 - S 50 -

- Paul Choi, et al. Primary splenic lymphoma presenting as splenic abscess - Figure 2.

Figure 3. (, ) Eight months later, abdominal CT showed progression of the now 5 8.

종내출혈이동반된소견으로이전보다악화된소견을보였다 ( 그림 3). 비장의원발성악성종양의가능성을생각하고확진및치료목적으로비장절제술을시행하였다. 적출한비장은 16.5 11 11 cm의크기를가지면서, 무게가 674.

면역조직화학적검사에서 CD20 강양성, CD30 양성을보였고, 한편 CD3, CD138, EM, Vimentin, CD34, ctin, Desmin 에는음성을보여 림프구기원의미만성대세포형림프종 (diffuse large cell lymphoma)

3 - Paul Choi, et al. Primary splenic lymphoma presenting as splenic abscess - Figure 2. (, ) Seven days after percutaneous drainage of the splenic cystic mass, abdominal CT showed no gross change in the splenic cystic mass. Figure 3. (, ) Eight months later, abdominal CT showed progression of the now cm cystic mass in the posterosuperomedial aspect of the spleen with some internal hemorrhage and a fatty liver. 종내출혈이동반된소견으로이전보다악화된소견을보였다 ( 그림 3). 비장의원발성악성종양의가능성을생각하고확진및치료목적으로비장절제술을시행하였다. 적출한비장은 cm의크기를가지면서, 무게가 g이었고, 비장내에비교적경계가명확한이미파열된창백한노란색을띄는 cm 크기의융기하는종양과함께광범위한괴사소견이관찰되었다 ( 그림 4). 비장조직에대한광학현미경검사에서비장림프종으로진단되었다 ( 그림 4). 면역조직화학적검사에서 CD20 강양성, CD30 양성을보였고, 한편 CD3, CD138, EM, Vimentin, CD34, ctin, Desmin 에는음성을보여 림프구기원의미만성대세포형림프종 (diffuse large cell lymphoma) 으로진단하였다. 환자는다른 장기나림프절의병변없이유일하게비장에국한된림프종의소견을보여원발성비장림프종으로확진할수있었다. 환자는증상호전후내원 14일째퇴원하였다. 고찰비호지킨림프종 (non-hodgkin s lymphoma) 의약 34~40% 가비장을침범하는것으로알려져있으며원발성비장림프종은비장을침범한전체림프종의 1% 이하로보고된다 1). 비장의원발성림프종의정의에대해서는여전히논란이있지만 DasGupta 등의정의에의하면비장비대가있으면서비장및비문림프절외에는다른장기의림프종이나림프절이없고비장절제술후 6개월이상재발이없어야한다고하였으며 2), Skarin 등은비장을침범하는림프종중에서비 - S 51 -

- 대한내과학회지 : 제 76 권부록 1 호통권 2009 - Figure 4. () Grossly, the spleen measured 16.5 11 11 cm and weighed 674.5 g. previously ruptured pale-yellow bulging mass measuring 12 11 7.5 cm was noted.

본증례에서는비장림프종외에는다른장기및림프선의침범이없었으며비장절제술후재발소견이없는것으로보아 DasGupta의정의에합당하다고할수있다. 원발성비장림프종의가장흔한임상증상은좌상복부동통이며체중감소, 비장비대, 피로감등도흔하며, 체중감소, 열, 야간발한 (night sweat) 같은 증상을보이기도한다 1,4-7).

4 - 대한내과학회지 : 제 76 권부록 1 호통권 Figure 4. () Grossly, the spleen measured cm and weighed g. previously ruptured pale-yellow bulging mass measuring cm was noted. On sectioning, the lesion showed extensive necrosis. () Microscopic histology revealed diffuse large cell lymphoma (H&E stain, 400). 장비대가현저하다면원발성비장림프종으로정의할수있다고하였다 3). 본증례에서는비장림프종외에는다른장기및림프선의침범이없었으며비장절제술후재발소견이없는것으로보아 DasGupta의정의에합당하다고할수있다. 원발성비장림프종의가장흔한임상증상은좌상복부동통이며체중감소, 비장비대, 피로감등도흔하며, 체중감소, 열, 야간발한 (night sweat) 같은 증상을보이기도한다 1,4-7). 또한혈액학적으로는빈혈, 백혈구감소증, 혈소판감소증및적혈구침강속도의증가의소견을보이는경우도있다 1,5). 본증례의환자에서는좌상복부통증외에는다른증상이없었으며혈액검사에서는경도의백혈구증가소견만있었다. 이는비장종괴의크기증가나비장기능의항진으로인한증상이발견하기전에발견되었기때문인것으로생각된다. 원발성비장림프종의진단은임상증상뿐만아니라복부초음파검사나복부전산화단층촬영등의방사선소견이도움이된다 8-11). 원발성비장림프종의복부초음파소견은대부분저에코로나타나며드물게석회화 (calcification) 또는가스때문에밝은에코를보일수도있다 9). 무에코 (anechoic) 부위는액화성괴사 (liquefactive necrosis) 를나타내기도하는데열이동반되어있는경우는비장농양과감별이힘들수있다 9). 원발성비장림프종의 CT 소견은조영제증강촬영에서대부분저음영으로보이며매우저음영인부위는괴사나저혈관분포를가진고형종양을나타낼수있다 9). 특히대세포 형비장림프종의경우는대부분 CT에서저음영의크기가큰단발성또는다발성비장종괴로나타나는경우가많아혈종, 단순낭종, 경색, 혈관종, 과오종, 전이성악성종양뿐만아니라특히비장농양과감별해야한다 8,9,11,12). 비장농양의 CT 소견도때때로테두리조영 (rim enhancement) 을가진균질의저음영의병변을보이므로특히비장림프종과감별이어렵다 13,14). 본증례에서도복부초음파및 CT에서경계가명확한저음영의비장낭종성종괴로나타나면서종괴내에액화성괴사가동반되어있어처음진단시비장농양으로오인된경우로생각된다. 방사선학적으로감별이어려운비장종양이나원인을알수없는비장비대의최종적인진단을위해서는경피적가는바늘흡인생검술 (fine-needle aspiration biopsy) 같은조직검사를시행해야한다 15). 만약, 여러검사들에도불구하고진단이되지않으면탐색개복술 (exploratory laparotomy) 이나비장절제술을시행해야한다. 본증례에서는환자의나이가젊고처음비장농양의진단하에시행한경피적배액술에서농양이배액되었고, 배액세포검사에서악성세포가발견되지않았지만확진을위해가는바늘흡인생검술이나개복술을시행하려하였으나임상증상의호전으로인해더이상의침습적검사나치료를환자가거부해서시행하지못한점이확진이지연된한이유라고생각한다. 비장을침범하는림프종의육안병리학적소견을 hmann 등은종괴가없는균질한비장비대 (homogeneous enlargement without masses), 속립성종괴 (miliary masses), 다발성종 - S 52 -

5 - 최바울외 6 인. 비장농양으로보인원발성비장림프종 - 괴, 큰단발성종괴 (large solitary mass) 의네가지로분류했는데, 특히크기가크면서때때로괴사를동반한종양의경우는대세포형림프종이라고하였다 8,9,16). 본증례의경우도액화성괴사를동반한큰단발성종양의소견을보였으며조직검사에서대세포형림프종으로진단되어 hmann 등의보고와일치하였다. 비장의원발성림프종은대개비호지킨림프종이며주된현미경적조직학적유형은 림프구기원의소림프구형 (small lymphocytic type) 또는대세포형 (large cell type) 이다 3-6). 본증례의경우도 림프구기원의미만성대세포형림프종이었다. 원발성비장림프종의치료는복강경비장절제술 (laparoscopic splenectomy) 17) 또는비장절제술이확진및치료를위해효과적인방법이며국소방사선치료나복합화학요법등을병행하기도한다 1,6,18). 비장과비문림프선에국한된원발성비장림프종은비호즈킨림프종 I기 (stage I) 와비슷한좋은예후를가지는것으로보고되고있다 19). 요 비장의종괴발견시에비장농양, 단순낭종, 혈관종, 과오종등의감별진단뿐만아니라, 드물지만원발성비장림프종의감별이반드시필요하다. 특히임상적, 방사선학적으로감별이어려운경우확진을위한조직검사또는수술을시행해야한다. 본증례는좌상복부통증을주소로내원후비장농양진단하에경피적비장배액술및항생제요법으로임상증상이호전되었던환자가 8개월뒤통증및방사선소견이악화되어비장절제술을시행한결과, 수술소견및조직검사에서원발성비장림프종으로확진한환자를경험하였기에문헌고찰과함께보고하는바이다. 약 중심단어 : 원발성비장림프종 ; 비장농양 REFERENCES 1) rox, ishinsky JI, erry G. Primary non-hodgkin lymphoma of the spleen. m J Hematol 38:95-100, ) DasGupta T, Coombes, rasfield RD. Primary malignant neoplasms of the spleen. Surg Gynecol Obstet 120: , ) Skarin T, Davey FR, Moloney WC. Lymphosarcoma of the spleen: results of diagnostic splenectomy in 11 patients. rch Intern Med 127: , ) Kraemer, Osborne M, utler JJ. Primary splenic presentation of malignant lymphoma and related disorders. Cancer 54: , ) Spier CM, Kjeldsberg CR, Eyre HJ, ehm FG. Malignant lymphoma with primary presentation in the spleen. rch Pathol Lab Med 109: , ) Folk S, Stutte HJ. Primary malignant lymphoma of the spleen: a morphologic and immunohistochemical analysis of 17 cases. Cancer 66: , ) Gobbi PG, Grignani GE, Pozzetti U, ertoloni D, Pieresca C, Montagna G, scari E. Primary splenic lymphoma: does it exist? Haematologica 79: , ) Meyer JE, Harris NL, Elman, Stomper PC. Large-cell lymphoma of the spleen: CT appearance. Radiology 148: , ) Dachman H, uck JL, Krishnan J, guilera NS, uetow PC. Primary non-hodgkin s splenic lymphoma. Clin Radiol 53: , ) Kamaya, Weinstein S, Desser TS. Multiple lesions of the spleen: differential diagnosis of cystic and solid lesions. Semin Ultrasound CT MRI 27: , ) Kim KH, Cho CK, Choo SW, Kim HJ, Kim KS. Primary lymphoma of the spleen: a case report. J Korean Surg Soc 52: , ) Kweon WJ, ae JS, Song IS. Hamatoma of the spleen: a case report. J Korean Surg Soc 58: , ) Debeuckelaere S, Schoors DF, uydens P, Du Ville LD, Spapen HD, Reynaert HM, De Vis G. Splenic abscess: a diagnostic challenge. m J Gastroenterol 86: , ) Sarr MG, Zuidema GD. Splenic abscess: presentation, diagnosis and treatment. Surgery 92: , ) Ramdall R, Cai G, lasio TM, Levine P. Fine-needle aspiration biopsy for the primary diagnosis of lymphoproliferative disorders involving the spleen. Diagn Cytopathol 34: , ) hmann DL, Kiely JM, Harrison EG Jr, Payne WS. Malignant lymphoma of the spleen. Cancer 19: , ) Choi Y, Lee CY. Laparoscopic splenectomy for splenic tumors. J Korean Surg Soc 61: , ) Morel P, Dupriez, Gosselin, Fenaux P, Estienne MH, Facon T, Jouet JP, auters F. Role of early splenectomy in malignant lymphomas with prominent splenic involvement (primary lymphomas of the spleen): a study of 59 cases. Cancer 71: , ) Kehoe J, Straus DJ. Primary lymphoma of the spleen: clinical features and outcome after splenectomy. Cancer 62: , S 53 -

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Kbcs002.hwp Does Real-time Compound Imaging Improve Evaluation of reast Cancer Compared to Conventional Sonography? o Kyoung Seo, M.D., Yu Whan Oh, M.D., Kyu Ran Cho, M.D., Young Hen Lee, M.D., Hyung Joon Noh, M.D.,