슬라이드 1

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서진 채
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1 GI Lymphomas 성균관대학교의과대학내과이준행

3 모든사람은죽는다. 모든사람은세금을내야한다. 림프종분류는항상변한다.

4 Biologically rational classification morphology immunophenotype genetic features clinical features Clinically useful classification clinical features natural history prognosis treatment

5 Immunohistochemical markers B-cell: CD20, CD79a T-cell: CD3, CD45RO NK-cell: CD56 Ki-67: Burkitt lymphoma > DLBL > others Mantle cell lympoma: cyclin D1 HTLV-1 serology TdT, CD4, CD5, CD8, CD10, CD30, Bcl-2, cytokeratin AE1/AE3,etc

6 Rappar- port 1966 Kiel 1978 Working Formulation 1982 REAL 1994 WHO 2001 Revised WHO 2008

8 B-cell neoplasms of GI tract MALT lymphoma Diffuse large B-cell lymphoma (DLBCL) Follicular lymphoma Mantle cell lymphoma Burkitt lymphoma

9 T-cell neoplasms of GI tract 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

10 위장관림프종의분포 55-65% 20-35% 7-20%

11 Non-Hodgkin s lymphoma of the GI tract - Danish Lymphoma Study Group d Amore. JCO ;12:

12 위장관림프종진단의임상요점 내시경진단이항상쉬운것은아니다. 병리검사결과해석에주의하자. 우리나라에는장 T-세포림프종이많다. 진단이지연될수있다. 위장관림프종은매우다양하다.

14 Mantle zone lymphoma Follicular lymphoma Burkitt s lymphoma Chronic lymphocytic leukemia Small lymphocytic lymphoma Waldenström s macroglobulinemia Sézary syndrome Mycosis fungoides Peripheral T cell lymphoma

15 stem cell lymphoid progenitor progenitor-b ALL pre-b immature B-cell CLL mature naive B-cell germinal center B-cell DLBCL, FL, HL memory B-cell MM plasma cell

16 내시경진단이 항상쉬운것은아니다. 성균관대학교의과대학내과이준행

17 Endoscopic diagnosis of GI lymphoma Relatively low incidence Variable endoscopic findings in the particular type of lymphoma. BUT, think about the gastric adenocarcinoma.

18 Diagnosis of ulcerative lesions EGC AGC AGC DLBL DLBL GIST

19 This extensive DLBCL expands well.

20 This stomach with DLBCL did not expand well by air infusion.

21 In most DLBL cases, we don t need to evaluate the finding of air expansion.

22 Diffuse type??? Taal. Gut 1996;39:

23 Fold thickening by lymphoma 부산대학교김광하

24 Capsule endoscopy for GI lymphoma Flieger. Endoscopy 2005;37:

25 Recurrent duodenal bleeding - Extronodal marginal zone lymphoma of MALT

26 Follicular lymphoma 자료제공 : 김미진

27 병리검사해석에주의하자. 성균관대학교의과대학내과이준행

28 Make friends with a pathologist There is lymphoid hyperplasia with mild to moderate cellular atypism, but these findings are not sufficient for the pathologic diagnosis of GI lymphoma.

29 우리나라에는 장 T- 세포림프종이많다. 성균관대학교의과대학내과이준행

31 T-cell 17.9% B-cell 82.1% Kim YH, Lee JH, et al. Digest Dis Sci 2005;50:

32 Endoscopic findings Classification B cell (n=63) T cell (n=15) All (n=78) Fungating 34 (54.0%) 2 (13.3%) 36 (46.2%) Ulcerative 3 (4.8%) 7 (46.7%) 10 (12.8%) Infiltrative 5 (7.9%) 0 5 (6.4%) Ulcerofungating 17 (27.0%) 1 (6.7%) 18 (23.1%) Ulceroinfiltrative 4 (6.3%) 5 (33.3%) 9 (11.5%) * P < Kim YH, Lee JH, et al. Digest Dis Sci 2005;50:

33 B cell T cell Kim YH, Lee JH, et al. Digest Dis Sci 2005;50:

34 진단이지연될수있다. 성균관대학교의과대학내과이준행

35 Delayed diagnosis of colon lymphoma was seen in T cell lymphomas (n=6, 35.3%) Age /Sex Symptoms Initial impression Initial management Time to final diagnosis 24/M Abdominal pain UC with perforation Persistent Sx after operation 3 months 66/F Frequent loose stool UC Persistent Sx after steroid 13 months 45/M Hematochezia Intestinal tuberculosis Weight gain after anti-tbc 12 months 67/M Frequent loose stool Intestinal tuberculosis Weight gain after anti-tbc 6 months 33/M Abdominal pain Crohn s Disease Medication 15 months 30/M Diarrhea r/o amebiasis Medication 6 months Kim YH, Lee JH, et al. Digest Dis Sci 2005;50:

36 Chronic diarrhea, loss of weight (15 kg) and night sweat (F/43) duodenum ileum Final diagnosis: peripheral T cell lymphoma

37 Sudden abdominal pain ileocecal intussusception was diagnosed

38 다양한위장관림프종증례 성균관대학교의과대학내과이준행

41 DLBCL of the stomach

42 DLBCL of the duodenum

43 DLBCL of the small bowel

44 Gastroduodenal involvement of diffuse large B cell lymphoma

47 Lymphoepithelial lesions Monocytoid B-cells

48 High grade MALToma??? MALT lymphoma without high grade component Extranodal marginal zone B cell lymphoma of MALT MALT lymphoma with high grade component DLBCL with a MALT lymphoma component DLBCL DLBCL without a MALT lymphoma component

49 The criteria is analogue. Score Diagnosis 0 Normal 1 Chronic active gastritis Chronic active gastritis with florid lymphoid follicle formation Suspicious lymphoid infiltrate, probably reactive Suspicious lymphoid infiltrate, probably lymphoma 5 MALT lymphoma Histological features Scattered plasma cells in lamina propria. No lymphoid follicles. Small clusters of lymphocytes in lamina propria. No lymphoid follicle. No lymphoepithelial lesions. Prominent lymphoid follicles with surrounding mantle zone and plasma cells. No lymphoepithelial lesions. Lymphoid follicles surrounded by small lymphocytes that infiltrate diffusely in lamina propria and occasionally into epithelium. Lymphoid follicles surrounded by marginal zone cells that infiltrate diffusely in lamina propria and into epithelium in small groups. Presence of dense infiltrate of marginal zone cells in lamina propria with prominent lymphoepithelial lesions.

50 What for Wootherspoon 3 and 4??? Choi MK. Korean J Gastroenterol 2011;57:

51 Personal protocol for MALToma Low-grade MALT lymphoma in the first endoscopic biopsy Staging work-up including EUS, CT, BM Stage E-I 2, II, III, IV or H. pylori (-) or high-grade HPE or ChemoRx H. pylori eradication (PCA 2 weeks ) + 2nd eradication, if necessary UBT 4-6 wks after completing antibiotic treatment Endoscopy, 3 months after completing eradication

52 Initial EGC 2 months later 6 months later

53 (HPE 1 개월 ) (HPE 3 개월 ) (HPE 5 개월 )

54 MALToma

55 MALToma

56 Plasmacytoma 로수술권유를받은후의뢰되었던 plasma cell predominant MALToma

57 RT 후호전된 MALToma

58 제균하였으나용종없어지지않아서용종절제술후경과관찰함

59 Recurrent duodenal bleeding - Extronodal marginal zone lymphoma of MALT

60 Marginal zone cells (IgM) Mantle zone cells (IgM+D) Follicle center cells (IgM, IgG, IgA or IgE, not IgD)

62 Mantle cell lymphoma Widespread adenopathy and frequently have bone marrow and extranodal involvement Most common type in GI tract: lymphomatous polyposis Pathologically low-grade, clinically highgrade

63 Lymphomatous polyposis (M/66)

64 Mantle cell lymphoma - stomach and colon, Cyclin D1: Positive

65 GI involvement of nodal MCL

66 SMC experience of 19 GI MCLs Presenting symptoms: abdominal pain (36.8%), GI bleeding (26.3%) Location: colon alone (47.4%), colon and stomach (36.8%), stomach alone (10.5% Endoscopy: polypoid (48.1%), infiltrative (33.3%), ulcerative (14.8%), fungating (3.7%) Kim JH. Acta Haematol 2012;127:129-34

68 Burkitt lymphoma at SMC, Burkitt s lymphoma n=80 Adult (age >18) n=47, 58.7% Child n=33, 41.3% GIT involve n=20, 43% No GIT involve n=27, 57% Jung & Lee. Korean J Gastrointest Endosc 2008:37-13

69 Involved organs of GI Burkitt lymphoma Organs Number (%) Stomach 11 (55%) Colon 4 (20%) Stomach + Duodenum 3 (15%) Stomach + Colon 1 (5%) Stomach + Duodenum+Colon 1 (5%) Jung & Lee. Korean J Gastrointest Endosc 2008:37-13

70 Fungating type Ulcerative type Ulcerofungating type Ulcerinfiltrative type Jung & Lee. Korean J Gastrointest Endosc 2008:37-13

71 Cumulative survival rate (%) Survival of GI Burkitt lymphoma Follow-up duration (months) Jung & Lee. Korean J Gastrointest Endosc 2008:37-13

72 Peripheral T cell lymphoma (PTCL) - CD3 (+), CD56(-)

73 PTCL detected during screening

74 PTCL detected during screening CD3 CD20

76 Kim JH, Lee JH. Endoscopy 2007;39:

77 Kim JH, Lee JH. Endoscopy 2007;39:

78 Kim JH, Lee JH. Endoscopy 2007;39:

79 GI NK/T-cell lymphoma at SMC Aggressive clinical course Frequently accompanied by extensive necrosis and perforation Common presenting symptom: GI bleeding Endoscopic pattern :ulceroinfiltrative (36%), ulcerative (27%) or superficial/erosive (27%) Kim JH, Lee JH. Endoscopy 2007;39:

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

81 제 16 회 SGEA 성균관대학교의과대학내과이준행

82 Situs inversus Retroflection 에서위치잡기가어려움 fundus antrum 찌글어진십이지장구부

83 Difficult insertion due to situs inversus

84 Situs inversus 사진제공 : 성영경선생님

85 Situs inversus Clin Gastroenterol Hepatol 2012;10:e33

86 Complete situs inversus is a rare congenital anomaly that results in a complete left-right inversion of the viscera. Although there is a link to various associated malformations and the immotile cilia or Kartagener syndrome, most patients with situs inversus have completely normal lives. Nevertheless, knowledge of this conditions is of paramount importance for the patient and the treating physician, as many gastrointestinal disease processes will manifest differently, ie, with pain on the wrong side. If an endosocpy is necessary, such as in our patient, careful attention should be paid to scope advancement. A useful maneuver is the endoscopic mirror technique. This technique mandates that all endoscopic maneuvers are performed inversely, as during a normal procedure. The basic principle of this technique is to perform the procedure as if the endoscopic exploration were the reflection of a standard endoscopy.1once the cardia is reached, a left lateral deflection of the tip of the scope is followed by advancing the scope to the left instead of to the right. The mirror changes during scope advancement should also be paralleled by manipulation of the handle wheels, ie, the lateral and up-and-down movements of the tip of the scope should also follow a mirror technique. This is especially important when the scope is inside the duodenal bulb. Clin Gastroenterol Hepatol 2012;10:e33

87 EMR for EGC in a dextrocardia patient 2 years later

88 아. 사진을잘남겼더라면 (1/3) Outside endoscopy: 위치는불명확. 정보는 antrum. 사진은무척애매. 그러나전정부소만같음. Outside pathology: focal high grade dysplasia Readoutside slide: atypical regenerating glands 이상황에서병소의위치가명확하였다면어떻게하시겠습니까? 만약위치가명확하였다면 atypical 은절반이상은 neoplastic 이므로 ESD 를바로시행할것임. 그러나위치가명확하지않다면내시경재검을해볼수밖에없음. 한번조직검사를한이후에즉시조직검사를하면잘나오지않는다는한계가있더라고재검하지않고 ESD 를할수는없는일임

89 아. 사진을잘남겼더라면 (2/3) 2nd endoscopy: 위치는대강알겠는데조직검사에서안나옴 (chronic gastritis) 심증은있으나물증이없는상태에서함부러 ESD 를할수도없음. Chronic gastritis 라는병리로 ESD 를하는것은규정위반임. 할수는있으나비보험이고이를설명하는것은무척어려움. 나중에암으로나와도비보험을보험으로바꿀수없기때문에더더욱어려움. 심평원의형편없는기준때문임. 3rd endoscopy with biopsy: adenocarcinoma 이제서야 ESD 를할수있음. 결국처음내시경을하였을때병소의위치, 크기, 모양에대한정보가얼마나중요한지를보여줌.

90 아. 사진을잘남겼더라면 (3/3)

91 경비내시경발견위암

92 Lymphoepithelioma-like carcinoma - SM invasion 1600 um

93 2 번 atypical gland with HGD Adenocarcinoma, M/D

94 Jung. Korean J Helicobacter Up Gastrointest Res 2013;13:

95 Mallory-Weiss tear and Pneumomediastinum by ESD

96 Jang. Korean J Helicobacter Up Gastrointest Res 2013;13:

97 Kim ( 대전선병원 ). Clin Endosc 2013;46:

98 경청해주셔서감사합니다.

슬라이드 1

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