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[Mantle cell lymphoma]

1. Mantle zone은 어디인가? Mantle cell lymphoma의 족보는?

2. Mantle cell lymphoma의 임상적 특징

가장 중요한 특징은 처음 발견시 systemic disease인 경우가 대부분이라는 것입니다. 위장관도 대부분의 경우에 이미 침윤되어 있다고 합니다 (World J Gastroenterol. 2010).

1) Subtype of B cell NHL

2) Typically present with widespread adenopathy and frequently have bone marrow and extranodal involvement (Lymph nodes > 90%, Bone marrow 70-80%, Spleen 60%, Liver 30%, GI tract 20-30%), Peripheral blood (lymphocytosis) 20-30%)

3) GI tract involvement: 과거에는 10-20% 정도로 생각하였으나 최근에는 80-90%로 보고 있음

4) Most common type in GI tract: lymphomatous polyposis

좌측 사진을 보십시오. 지금까지 제가 본 증례 중 가장 전형적인 lymphomatous polyposis 형태의 mantle cell lymphoma입니다.

5) Pathologically low-grade, clinically high-grade

6) 3-10% of all lymphoma cases

7) Mean age at presentation: 60 years

8) M:F = 3:1

9) Stage III-IV at presentation (70-80% of cases)

10) Poor prognosis: 5 years OS ≤ 30%

11) Best treatment modality: uncertain

3. Mantle cell lymphoma of the GI tract

1) Most common site: ileo-cecal region

2) Any other area may be involved: from the stomach to the rectum

3) Most patients have extensive nodal and bone marrow disease at presentation

4) Symptoms: abdominal pain, diarrhea, hematochezia --> non-specific

4. Colon involvement of MCL : lymphomatous polyposis type

좌측 사진을 보십시오. 지금까지 제가 본 증례 중 가장 전형적인 lymphomatous polyposis 형태의 mantle cell lymphoma입니다.

2016-9-23 내시경 집담회 서울대병원 증례. 대장 용종 EMR 후 cyclin D1 (+) mantle cell lymphoma로 진단되었으나 수 년 간 follow up loss가 되었음. 수 년 후 추적내시경에서 SMT 유사한 lymphomatous polyposis 양상이었음. 첫 임상상이 대장 용종이었고 10년 후까지 주된 병소가 대장과 mesentery였기 때문에 primary colonic mantle cell lymphoma로 판단하였음.

8년전 진단되었고 항암치료 후 호전되었다가 최근 재발됨. 용종 부위조직검사에서 모두 mantle cell lymphoma가 나옴.

5. Colon involvement of MCL : non- lymphomatous polyposis type

체중감소를 주소로 내원한 60대 여성의 대장내시경입니다. 조직검사에서 mantle cell lymphoma, cyclin D1 (+)가 나왔습니다. CT에서 ileum이 두꺼워 보였습니다. 경부 림프절과 골수 침윤이 있었습니다.

6. Stomach involvement of MCL

명란젓처럼 생겨서 깜짝 놀랐습니다.

9년 전 tonsil의 mantle cell lymphoma로 chemotherapy + RT 받은 환자의 위 내시경입니다. 조직검사에서 mantle cell lymphoma가 나와 stomach RT를 시행하였습니다.

7. GI involvement of nodal MCL (Salar A. Am J Surg Pathol 2006;30(10):1274-80)

1) Only 26% of patients presented with GI symptoms at the time of diagnosis.

2) MCL was present histologically in the lower GI tract of 53 of 60 patient (88%) and in the upper GI tract of 28 of 58 patients (43%).

3) Microscopic evidence of MCL was found in 84% of patients with normal visual (macroscopic) findings by lower endoscopy and in 45% of patients with macroscopically normal findings by upper endoscopy.

4) Abnormal mucosa was identified in 38% of cases by upper endoscopy (mainly mild nonspecific gastritis) and in 54% of cases by lower endoscopy (mostly micropolyps).

Duodenum, stomach and colon involvement of MCL

5) Histologically, infiltration by MCL was demonstrated in the stomach in 77% of cases and in the colon in 77% of cases.

6) As a whole, 92% of patients showed upper or lower GI tract infiltration by MCL.

8. GI mantle cell lymphoma, experiences at Samsung Medical Center (Kim JH. Acta Haematol 2012)

Endoscopic findings in mantle cell lymphoma with gastrointestinal tract involvement.

BACKGROUND AND AIMS: Mantle cell lymphoma (MCL) of the gastrointestinal (GI) tract is a rare disease with a poor prognosis. The aim of this study was to determine clinical and endoscopic characteristics of patients with GI MCL.

METHODS: Clinical features of 19 patients with GI MCL were reviewed along with the endoscopic findings on 27 anatomical lesions.

RESULTS: The initial presenting symptoms were abdominal pain (n = 7, 36.8%), GI tract bleeding (n = 5, 26.3%), dyspnea (n = 2, 10.5%), indigestion (n = 1, 5.3%), diarrhea (n = 1, 5.3%), cervical lymphadenopathy (n = 1, 5.3%), tonsilar mass (n = 1, 5.3%), and no symptoms (n = 1, 5.3%). On endoscopy, in 19 patients with 27 lesions, the anatomic locations of the lesions were: stomach, n = 2 (10.5%); stomach and colon, n = 7 (36.8%); terminal ileum and colon, n = 1 (5.3%); colon, n = 9 (47.4%). There was 1 fungating case (3.7%), 4 ulcerative cases (14.8%), 9 infiltrative cases (33.3%), and 13 polypoid cases (48.1%).

CONCLUSIONS: The endoscopic findings in GI MCL are variable, with common presenting manifestations of abdominal pain and GI bleeding.

9. Pathology of MCL

1) MCL is characterized by neoplastic expansion of the mantle zone surrounding lymph node germinal center, with a homogeneous population of small lymphoid cells displaying slightly to markedly

2) irregular nuclear outlines, small nucleoili and scant cytoplasm.

3) In contrast to follicular lymphomas, very few large cells are identified among the mantle cells. The malignant cells are intermediate in size, between those of small lymphocytic lymphoma and

4) hose of follicular small cleaved cell lymphoma.

5) Small cell (small lymphocyte-like; intermediate lymphocyte) 80% of cases

6) “Blastoid” (classical/monomorphic; pleomorphic) 20% of cases

7) B marker: IgM (+) or IgD(+), CD19, CD20, CD22, CD79

8) T marker: CD10(-), CD23 (-), CD5 (weakly +)

9) Cyclin D1 (+)

10) BCL6 negative

10. Genetics of MCL

1) t(11;14)(q13;q32)

2) Immunoglobulin (H and L chains) clonal rearrangement

3) IgVH unmutated (70-80% of cases) origin from a subset of (CD5+) “naive” (pre-germinal centre) B lymphocytes

4) Conventional cytogenetics : often complex caryotype; del 6q, del 11q22, del 13q14…

5) CGH : chromosomal gains (3q26-27,…); and losses (13,Y,…)

© 일원내시경교실 바른내시경연구소 이준행. EndoTODAY Endoscopy Learning Center. Lee Jun Haeng.