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Marginal zone lymphoma with a prominent blastic component (6121)
Marginal zone lymphoma with a prominent blastic componentclosed
Subtitle: B08-19005
Type:
lymph node
Sender:
ugnius
2008-07-10 19:13
INCTR - EBMWG Hematopathology Online
In 2004 DLBCL was diagnosed for 67 yrs old woman in lymph node (inguinal and neck lymphadenopathy, 7x3x3cm node). Appropriate therapy was applied.  
In 2007 tumor growth was noticed in thoracic subcutis (most probable lymph node with tumoral infiltration; 1,3x1,5x1,7cm).  
HISTO: on the slides.  
IH: CD20+; Bcl6/CD10(-)(rudimentary FC +); Mum1+; IgM+; IgD-; Ig kappa/lambda+ (without restriction); CD43/CD3(-); CyclinD1(-); Bcl2+. Ki67 ~50% in tumor (~30% in overall population). CD21/CD23+ FDC network desorgnized and focally proliferating.  
PRIMARY BIOPSY (PHOTOS "PRIMARY"): THE SAME PICTURE OF MZL. Missdiagnosed most probably due to large caliber of cells in diffuse areas.  
 
WORKING DIAGNOSIS: B cell lymphoma with FDC proliferation/follicle collonisation/formation and T cell enreachement (MzL?), transformed?  
QUEST: 1. CD10 and Bcl6 lost esp in diffuse zones, remains in destructed follicles. 2. Focal neovascularized AILT like picture. 3. DLBCL quality in diffuse zones.  
 
Thank you for beeing 2gether.
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tzankov
2008-07-11 14:27
I agree with your diagnosis of marginal zone lymphoma with follicular colonization. I do not seen enough evidence to call it trasnformed DLBCL, since there are no sheets of blasts, though I admit that there are lots of scattered blasts... we may call it "blast-rich" MZL, a designation that is popular in the german-speaking world.
ugnius
2008-07-11 16:39
Thank you.
torlakovic
2008-07-12 05:44
As there are some (or many) Bcl-6+ cells still present, could you demonstrate that these are clearly Bcl-2-negative? This would be essential to prove that this is colonization of benign GCs. Many times colonization is extensive to the degree that residual Bcl-6+ cells are either very scant or even entirely absent, but since they are still present, they need to be Bcl-2-. I cannot see that from your images.
hurwitz
2008-07-12 18:59
I agree the diagnosis of MZL with follicular colonialization is the most probable one. I also agree that the findings do not meet the full criteria for full high grade transformation, however the amount of blasts is high. If possible a comparison of the amount of large cells between the previous biopsy (2004) and the present one is indicated to asses the degree of tumor progression in the interval. Are staging bone marrow biopsies routine in your country ?
Went
2008-08-07 10:37
I agree with the precedent speakers: nodal MZL with high amount of blasts, not fulfilling the criteria of fullblown (secondary) DLBCL.  
In the new WHO classification to be published this year, as far as I know,there will not be an entity blastic MZL, therefore the in my opinion at times very difficult decision, when to diagnose secondary DLBCL ex MZL will remain difficult. This is illustrated by this case, where there is a high amount of blastic B-cell, but these do not form sheets. Ki-67 can be helpful to identify areas of high proliferation and may transformation.
ugnius
2008-08-08 09:14
Thank you once more. PRIMARY photos is taken from primary tumor with the similar histopicture.
hurwitz
2008-08-08 16:39
 
Please regard Dr.Went's comment as the final diagnosis.  
 
Thanks to the submitter and to the participants in the discussion
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Last modified: 2008-07-10 19:13:45