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아라 봉
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1 Gastro intestinal lymphomas R3. 정세진

2 정의 GI lymphoma introduction 위장관및이와관련된배액부위림프절에국한, 간이나비장및기타림프절침범이없고, 단순흉부촬영상이정상, 말초혈액검사의백혈구가정상인경우. Extranodal lymphoma 69.6% GI lymphoma 29.22% Kim JM, WHO Classification of Malignant Lymphomas in Korea: Report of the Third Nationwide Study, The Korean Journal of Pathology 2011; 45:

3 2001 년 WHO Lymphoma classification 형태학적소견, 세포의기원 ( 면역표현형으로결정 ), 임상소견과유전자형 ( 핵형, 바이러스유전자존재 ) 까지고려한새로운분류를제안 * 호지킨림프종 : 매우드뭄

4 GI Lymphoma classification B-cell neoplasms MALT lymphoma Diffuse large B-cell lymphoma (DLBCL) Follicular lymphoma Mantle cell lymphoma Burkitt lymphoma T-cell neoplasms Adult T-cell leukemia/lymphoma (HTLV-1+) Enteropathy-type intestinal T-cell lymphoma Anaplastic large cell lymphoma, T- or null cell type NK/T-cell (angiocentric) lymphoma Peripheral T-cell lymphoma, unspecified

5 Relative frequency of the histologic subtypes in NHL Kim JM, WHO Classification of Malignant Lymphomas in Korea: Report of the Third Nationwide Study, The Korean Journal of Pathology 2011; 45:

6 GI lymphoma distribution Stomach 68 to 75 percent Small bowel (including duodenum) 9 percent Ileo-cecal region 7 percent Rectum 2 percent Diffuse colonic involvement 1 percent More than one GI site 6 to 13 percent

7 Clinical presentation and diagnosis 대에호발, 남 = 녀 초기 : 비특이적소화불량 검진목적내시경에서발견되는경우가많음 진행 : 복통, 위장관출혈, 체중감소, 복부종괴 주로전정부에호발하지만체부및분문에서도발생 내시경에서는모든위치에서다양한형태

8 Endoscopic diagnosis Variable endoscopic findings in the particular type of lymphoma. BUT, think about the gastric adenocarcinoma! Close consultation to the pathologist is the key. Endoscopic findings described in the textbook (Yamada eds.) Gastric lymphoma can be grossly indistinguishable from adenocarcinoma, but is actually a diffusely infiltrating submucosal lesion. The tumor may present as a polypoid lesion with ulcerations, but like other submucosal lesions, the overlying mucosa may traverse the mass as a characteristic bridging fold. Lymphomas do not have a predilection for any particular region of the stomach, but are more likely than other cancers to present with diffuse infiltration and enlarged rugal folds.

9 림프종에특징적인내시경소견은없음 진단이지연될수있음 Endoscopic diagnosis 악성질환은틀림없는데선암의전형적인모양이아닐때, 종양크기 에비해 obstruction 이가벼울때, 다발성병소일때에는의심 림프종을의심할수있는소견에서도선암이나오는경우가있으므 로반드시조직검사확인이필요함

10 Polypoid lesion with ulcerations Lymphoma vs. AGC

11 Diffuse infiltration and enlarged rugal folds, Lymphoma vs.egc

12 EGC-like lesions

13 Diffuse type: similar to AGC B-IV

14 Lymphomatous polyposis

15 Multiple hemorrhagic & erosions

16 GI lymphoma staging Ann Arbor system Lugnao system Modified Ann Arbor system

17 Paris staging system 원발장기의심달도 & 림프절침범 & 원격장기의침윤 TNM staging 을근간

18 결론 : 위장관림프종 내시경진단이항상쉬운것은아니다. 병리검사결과해석에주의하자. 우리나라에는장 T-세포림프종이많다. 진단이지연될수있다. 위장관림프종은매우다양하다.

19 Gastro intestinal lymphomas -Maltoma

20 MALToma introduction 2008 WHO classification

21 Endoscopic diagnosis of MALToma Gastritis-like MALTomas, Multifocal atrophy pattern MALTomas Multinodular MALTomas Ulcerative MALTomas Polypoid MALTomas Early gastric cancer-like MALTomas. 실제로는약간지저분하면서심하고불규칙하게분포하는미란성위염과비슷한형태를보이는경우가가장많다.

22 Endoscopic diagnosis of MALToma

23 Histologic Scoring 정상적인 Peyer s patch 의림프조직을닮은반응성림프여포 Marginal zone/monocytoid B- 세포 Lymphoepithelial lesion 소림프구와형질세포침윤 (plasma cell infiltration) 과함께여포아세포 (centroblast) 와면역아세포 (immunoblast) 가출현

24 Treatment

25 Post Treatment Evaluation GELA staging system 이라는것이소개되어큰혼란 - pmrd (probable minimal residual disease) 가관해로해석 Treatment failure 점막하층을넘어침범 고도요소 내시경초음파에서위주위의림프절이침범 t(11;18)(q21;q21) 와같은염색체이상이존재하는경우 방사선치료 (30GY) CTx

26 Treatment Initial EGC 2 months later 6 months later

27 GI lymphomas 에대한이준행교수님의요약 1) 위장관림프종은점막하병소처럼보이는경우도있으나점막병소인경우가더많습니다. 2) 아래와같은경우에는림프종을의심하는것이좋습니다. 암은암같은데 adenocarcinoma의일반적인모양이아닌경우. 종양크기에비해 obstruction이가벼울때혹은잘펴질때 조직검사에서 "dense lymphoid infiltration 이나림프종으로진단하기어렵다 (not sufficient for the diagnosis of lymphoma) 와같은결과일때 짧은기간에병소가많이커졌을때 다양하고 bizzare한모양의병소가여러개있을때 IBD나기타염증성질환환자가일반적인치료에도호전이없을때

28 References. Endotoday Lee JY, Lee JH, Gastrointestinal Lymphoma, The Korean Journal of Helicobacter and Upper Gastrointestinal Research Cho SJ, Diagnosis and Staging of Gastrointestinal Lymphoma, 대한소화기내시경학회세미나, 제 55 회. Kim JM, WHO Classification of Malignant Lymphomas in Korea: Report of the Third Nationwide Study, The Korean Journal of Pathology 2011; 45: Steven H. Swerdlow, The 2016 revision of the World Health Organization classification of lymphoid neoplasm, BLOOD, 19 MAY 2016 x VOLUME 127, NUMBER 20

슬라이드 1

슬라이드 1 Gastric MALT Lymphoma 성균관대학교의과대학내과이준행 모든사람은죽는다. 모든사람은세금을내야한다. 림프종분류는항상변한다. Biologically rational classification morphology immunophenotype genetic features clinical features Clinically useful classification