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1 기관과대장에발생한원발성 mucosa-associated lymphoid tissue 림프종 1 예 순천향대학교의과대학내과학교실, 해부병리학교실 1, 진단방사선과교실 2 배준용, 나현식, 최재성, 최준호, 박성규, 장안수, 고은석 1, 백상현 2, 박재성 2, 박춘식 A Case of Primary Mucosa-Associated Lymphoid Tissue Lymphoma of the Trachea and Colon Jun Yong Bae, M.D., Hyun Sik Na, M.D., Jae Sung Choi, M.D., Jun Ho Choi, M.D., Sung Gyu Park, M.D., An Soo Jang, M.D., Eun Suk Ko, M.D. 1, Sang Hyun Paik, M.D. 2, Jae Sung Park, M.D. 2, Choon Sik Park, M.D. Department of Internal Medicine, Anatomical Pathology 1, Diagnostic Radiology 2, Soonchunhyang University, School of Medicine, Bucheon, Korea The 64-year-old female patient with cough and intermittent hemoptysis of six months duration visited our hospital. On chest computed tomography, a small, ovoid, 1.3cm sized and well enhanced lesion was detected on the distal trachea. Two multiple lobulated lesions on the sigmoid and transverse colon were revealed on the colonoscopy. The histological findings showed small and medium sized lymphocytes infiltration, CD20 and CD79a positive staining and multiple lymphoepithelial lesions on the distal trachea and colon tissues. Herein, a case of primary MALT lymphoma, with involvement of the trachea and colon, which was treated with rituximab (CD20 anti-monoclonal antibody), cyclophosphamide, adriamycin, vincristine and prednisolone (CHOP regimen), is reported. (Tuberc Respir Dis 2005; 59: ) Key words : Mucosa-associated lymphoid tissue, Trachea, Colon 서 Mucosa-associated lymphoid tissue 는안구, 침샘, 갑상선, 폐, 위, 소장, 대장, 방광, 여성생식기등에서존재하는림프조직으로림프절과형태와기능면에서매우비슷하다. Mucosa-associated lymphoid tissue 림프종 ( 이하 MALT 림프종 ) 은일반적인림프종이림프절에서면역세포중한종류가악성변화를가져오는것과비슷하게 mucosa-associated lymphoid tissue에서면역세포중한종류가악성변화를가지는것이다 1. 위장관의경우에있어서 MALT 림프종은역학, 병리기전및치료가잘정립되어있지만다른 This work was supported by a grant from the Korean Ministry of Health and Welfare, Republic of Korea (No. 01-PJ3-PG6-01GN04-003). Address for correspondence : Choon-Sik Park, M.D Jung-Dong, Wonmi-Gu, Bucheon-Si, Gyeonggi-Do, Seoul , Korea Phone : Fax : mdcspark@unitel.co.kr Received : Jun Accepted : Jul 론 장기의 MALT 림프종의경우매우드물기때문에비교적예후는좋은편이라고는하지만이에대한자세한임상경과, 치료및예후가드물게보고되어있다. 이에저자등은기관과대장에동시에발생한 MALT 림프종을경험하였기에보고하는바이다. 증례환자 : 64세여자주소 : 기침, 간헐적객혈현병력 : 내원 6개월전부터발생한기침과간헐적으로가래에피가묻어나오는정도의객혈을주소로내원함. 과거력 : 특이사항없었고흡연력도없었음. 가족력 : 특이사항없음. 진찰소견 : 생체징후는 130/80 mmhg, 체온 36.5 호흡수 20회 / 분, 맥박수 90회 / 분이었으며흉부청진에서양측전폐야에서비교적깨끗한호흡음들렸음. 림프절종대나간비종대는관찰되지않았다. 검사실소견 : 일반혈액검사및혈청화학검사에서 193

JY Bae et al.: A case of primary mucosa-associated lymphoid tissue lymphoma of the trachea and colon Figure 1. Computed tomography images (A. Axial scan, B.

10L( 예측치의 106%), FEV1/FVC 62% 를보였으며노력성호기류-용적곡선에서흉곽내폐쇄소견을보였다. 방사선소견 : 단순흉부촬영에서는특이소견없었으나흉부전산화단층촬영에서원위부기관의비교적조영이잘되는 1.3cm의난원형병변 (Fig. 1) 이관찰이되었다.

2 JY Bae et al.: A case of primary mucosa-associated lymphoid tissue lymphoma of the trachea and colon Figure 1. Computed tomography images (A. Axial scan, B. Coronal scan) show well enhanced, small ovoid lesion (about 1.3cm) on distal trachea. 특이소견없었다. β2-microglobulin의증가나다른종양표지자, HIV 감염이나자가면역질환의증거도없었다. 폐기능검사에서 FVC 3.36L( 예측치의 123%), FEV1 2.10L( 예측치의 106%), FEV1/FVC 62% 를보였으며노력성호기류-용적곡선에서흉곽내폐쇄소견을보였다. 방사선소견 : 단순흉부촬영에서는특이소견없었으나흉부전산화단층촬영에서원위부기관의비교적조영이잘되는 1.3cm의난원형병변 (Fig. 1) 이관찰이되었다. 기관지내시경으로관찰하였을때원위부기관에주위와비교하여점막의변화는없었으나기관내로표면이부드러운융기형병변 (Fig. 2) 이관찰되었다. 조직학적소견 : 경식기관지내시경하에서조직생검을시행하였으며조직검사에서작고중간크기의림프구의침윤을보이며기관상피는잘유지되었다. 면역화학염색에서 CD20과 CD79a에양성소견을보였으며 Anti-kappa와 lamda light chain 염색에서는단클론성을보이지않는 mucosa-associated lymphoid tissue 림프종소견이관찰되었다 (Fig. 3). 치료및경과 : 환자의기관지 MALT 림프종이전신적인질환이아님을증명하기위해골수조직검사, 두부및경부와복부컴퓨터단층촬영, 위와대장내시경, 혈액및뇨의단백질전기영동을시행하였다. 대장내시경에서 S자대장과횡행대장의소엽의용종 모양을보이는병변 (Fig. 4) 이관찰되었다. 대장조직검사에서도작은림프구의침윤과함께 CD20과 CD79a의양성을보였으며림프상피성병변이관찰되었다 (Fig. 5). 환자는 MALT 림프종으로병기는횡경막위아래의양측에병변을가지는 III E 로서 CD20 단클론항체인 rituximab 과 CHOP (cyclophosphamide, Doxorubicin, Vincristine, Prednisolone) 화학요법을시행하였고추적관찰중이다. Figure 2. Bronchoscopy shows smooth rounded lesion with normal mucosa on distal trachea. 194

Tuberculosis and Respiratory Diseases Vol. 59. No. 2, Aug. 2005 Figure 3.

CD20, CD79a co-stain ing) show the small and medium sized lymphocytes with CD20, CD79a positive staining.

Transverse colon) show multiple lobulated, smooth mar ginated lesions with normal mucosa.

3 Tuberculosis and Respiratory Diseases Vol. 59. No. 2, Aug Figure 3. Histologic findings of the tracheal specimens (A. Haematoxyline-eosin staining, B. CD20, CD79a co-stain ing) show the small and medium sized lymphocytes with CD20, CD79a positive staining. Figure 4. Colonoscopic findings(a. Sigmoid colon, B. Transverse colon) show multiple lobulated, smooth mar ginated lesions with normal mucosa. Figure 5 Histologic findings of the colonoscopic specimens (A. Haematoxyline-eosin staining, B. CD20, CD79a costaining) show the small and medium sized lymphocytes with CD20, CD79a positive staining, lymphoepithelial lesions. 195

4 JY Bae et al.: A case of primary mucosa-associated lymphoid tissue lymphoma of the trachea and colon 고찰 MALT 림프종은 Isaacson과 Wright 가 년처음으로위장관의저급 B 세포림프종으로보고하였다. MALT 림프종은침범부위가위장관인경우가대부분이고안구, 침샘, 방광, 소장, 대장, 폐, 갑상선, 여성생식기등도발생하는것으로알려져있다. MALT 림프종의조직학적소견은활성화된배중심 (germinal center) 형성, 불규칙한핵과투명한세포질을가지는중심세포 (centrocyte-like cell) 모양의세포, 림프상피성병변을만드는임파구의상피침윤을보이며일부에서대세포 (large cell) 를포함한다. 그리고면역조직화학검사에서특징적으로단클론의확장을보이는 B cell 림프종의형태를보이며 B 세포표지자인 CD20, CD19, CD79a에양성을보인다. 대부분의 MALT 림프종은외과적수술및방사선치료등의국소치료에우수한효과및예후를보이는것으로알려져있다. 이것은 MALT 림프종에서임파선의국소또는원격전이가드물며골수전이가잘일어나지않기때문이다. 원발성기관종양이드물기는하지만 Grillo과 Ma thisen은 2 대부분이편평세포암종과선양낭성암종 (adenoid cystic carcinoma) 으로보고하였다. MALT 림프종의기관에서의빈도는 Zinzani 등 3 의비위장관 MALT 림프종의연구결과를보면 75예중 1예가보고되어매우드문것으로생각된다. 또한기관을침범한 MALT 림프종의경우국소치료에대한효과가있는것으로보고하고있으나장기간의예후에대한통계는거의없다 4, 년 Maeda 등 6 이기관지림프종이외과적절제로만치료하여 5년이상의생존율을보고하였다. Okubo 등 7 은외과적절제만으로 53개월간재발이없었음을보고하였다. 위장관의 MALT 림프종은약 90% 의저급위 MALT 림프종이 stage I 이며 80-95% 의 5년생존율을가진다. 조기에발견된경우 Helicobacter pylori 치료만으로대부분이충분한치료효과를볼수있었으며 8-10, Lugano staging system을이용하여 stage I 과 stage II의경우에방사선치료 (30CGy) 는 100% 완전관해, 100% 에서재발없음을보고했다 11. Ann Arbor stage IE 17명과 stage IVE 7명을대상으로단일제제 (cyclophosphamide 또는 chlorambucil) 경구화학요법은 75% 의완전관해와 75% 의 5년생존율을가져왔다. 12 대장과갑상선에서도 MALT 림프종은외과적절제, 방사선치료, 화학요법이좋은결과를가져오는것으로알려져있다. MALT 림프종에서여러장기침범을보인경우가있는데 Thieblemont 등 13 은 MALT 림프종 108예중 35예 (32%) 에서다장기침범을보고하였다. Yoshino 등 14 이 7년간 MALT 림프종 304예를추적관찰하여 7 예에서 MALT 림프종이 2개이상장기를침범한경우가있었고, 7예중 6예가장의침범을포함하고있었다. 이와같이 MALT 림프종은대략 2% 에서 32% 까지다양한다장기침범이있으며다장기침범의경우에서장관을침범한경우가많음을알수있다. MALT 림프종의불량한예후가대세포 (large cell) 구성비, 다장기침범, 전신적인증상이연관이있을것이라생각을하지만아직정확한보고는없다. 따라서저자등은 MALT 림프종에서매우드문기관과대장을침범한 MALT 림프종을가진 1예를 CD20 의단클론항체인 rituximab 과 CHOP의화학요법으로치료하고추적관찰중인 1예를보고하는바이다. 요 MALT 림프종은안구, 침샘, 감상선, 폐, 위, 소장, 대장, 방광, 여성생식기에발생하며, 대부분이단일기관을침범하고국소치료와화학요법에좋은예후를보인다. MALT 림프종이기관을침범하는경우는거의보고된경우가없으며국내에선폐의 MALT 림프종이보고된적이있었다 15. 이에저자등은기관과대장에발생한원발성 MALT 림프종 1예를문헌고찰과함께보고하는바이다. 약 참고문헌 1. Isaacson P, Wright DH. Malignant lymphoma of mu cosa-associated lymphoid tissue: a distinctive type of B-cell lymphoma. Cancer 1983;52: Grillo HC, Mathisen DJ. Primary tracheal tumors: treatment and results. Ann thorac Surg 1990;49:

5 Tuberculosis and Respiratory Diseases Vol. 59. No. 2, Aug Zinzani PL, Magagnoli M, Galieni P, Martelli M, Poletti V, Zaja F, et al. Nongastrointestinal low-grade mu cosa-associated lymphoid tissue lymphoma: analysis of 75 patients. J Clin Oncol 1999;17: Kaplan MA, Pettit CL, Zukerberg LR, Harris NL. Primary lymphoma of the trachea with morphologic and immunophenotypic characteristics of low-grade B-cell lymphoma of mucosa-associated lymphoid ti ssue. Am J Surg Pathol 1992;16: Wiggins J, Shefield E, Green M. Primary B cell mali gnant lymphoma of the trachea. Thorax 1988;43: Maeda M, Kotake Y, Monden Y, Nakahara K, Kawa shima Y, Kitamura H. Primary malignant lymphoma of the trachea: report of a case successfully treated by primary end to end anastomosis after circumferential resection of the trachea. J Thorac Cardiovasc Surg 1981;81: Okubo K, Miyamoto N, Komaki C. Primary mucosaassociated lymphoid tissue (MALT) lymphoma of the trachea: a case of surgical resection and long term survival. Thorax 2005;60: Steinbach G, Ford R, Glober G, Sample D, Hagemeister FB, Lynch PM, et al. Antibiotic treatment of gastric lymphoma of mucosa-associated lymphoid tissue: an uncontrolled trial. Ann Intern Med 1999;131: Wotherspoon AC, Doglini C, Diss TC, Pan L, Moschini A, de Boni M, et al. Regression of primary low grade B-cell gastric lymphoma of mucosa-associated lymp hoid tissue type after eradication of Helicobacter pylori. Lancet 1993;342: Roggero E, Zucca E, Pinotti G, Pascarella A, Capella C, Savia A, et al. Eradication of Helicobacter pylori infection in primary low-grade gastric lymphoma of mucosa-associated lymphoid tissue. Ann Intern Med 1995;122: Schechter NR, Portlok CS, Yahalom J. Treatment of mucosa-associated lymphoid tissue lymphoma of the stomach with radiation alone. J Clin Oncol 1998;16: Hammel P, Haioun C, Chaumette MT, Gaulard P, Divine M, Reyes F, et al. Efficacy of single agent chemotherapy in low grade B-cell mucosa-associa ted lymphoid tissue lymphoma with prominent gastric expression. J Clin Oncol 1995;13: Thieblemont C, Bastion Y, Berger F, Rieux C, Salles G, Dumontet C, et al. Mucosa-associated lymphoid tissue gastrointestinal and nongastrointestinal lym phoma behavior: analysis of 108 patients. J Clin Oncol 1997;15: Yoshino T, Ichimura K, Mannami T, Takase S, Ohara N, Okada H, et al. Multiple organ mucosa-associated lymphoid tissue lymphomas often involve the intestine. Cancer 2001;91: Han MS, Kang DW, Choi GY, Lee YD, Cho YS. A case of primary extranodal marginal zone B-cell lymph oma of the MALT type. Tuberc Respir Dis 2003;54:

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Jkbcs016(92-97).hwp Expression of bcl-2 and Apoptosis and Its Relationship to Clinicopathological Prognostic Factors in Breast Cancer - A Study with Long Term Follow-up correlated with the survival rate.(journal of Korean