45 yrs male with recurrent orbital/lacrimal gland low grade B NHL.  
In 2014 on primary event called (by myself) as FL grade 2 (75% follicular)(Bcl6+ CD10-).  
 
in 2014 in CUTANEOUS THORACIC core biopsy changed into B MZL diagnosis (mainly interfollicular CD20+ IgM+ population with scattered nodules of Bcl6+: different from IgM+ nodules in 2021 relapse).  
 
In 2021 (ocular relapse) the same picture as in primary: close to B FL.  
 
IH (attenuated)(2021): CD20+; Bcl2+; Ig kappa+; IgM+ >> IgD+; MUM1/FoxP1+; LMO2+/-(weak); GCET1/Bcl6/CD10(-); CD5/CD43/CyclinD1/MNDA1/CD21/CD23/CD30(-); EBER(-); Ki67 index up to 35%.  
Bcl2 break (FISH): absent.  
MOLECS (clonal comparison): ND yet (old 2014 material).  
 
VULSK sonoscopy (2020 July): GEN LYMPHADENOPATHY  
RIGHT inguinal LN 9 x 5 mm, LEFT- 13 x 6 mm. LEFT NECK LN 13 x 6 mm, RIGHT- MULTIPLE up to 30 x 18 mm. RIGHT SUBCLAVICULAR LN iki 20 x 12 mm. AXILAR LN: normal.  
 
LDH 2020 (U/L) 197 normal (125-243)  
 
CT (2014): Subcutaneous masses left thoracis area and left inquinal zone mass and lymph node. Paraesophageal enlarged LN.  
PET (2014): the same.  
 
PROPOSAL: Systemic disease: B FL (in all 3 biopsies, not represented in skin biopsy (interfolicular area?) vs 2 diseases?  
 
PROBLEM: Bcl6+ CD10(-) and Mum1/LMO2/FoxP1+ immunophenotype...  
 
ThanYouforbeeingClose 2021.

tzankov 2021-01-27 08:48	Dear Ugnius,     difficulat case, indeed, yet I think it is a MZL. It started in ocular adnexa (typical for MZL), it spread to MALT-sites (typical for MZL), it relapsed in teh ocular adnexa (typical for MZL), it expresses onky BCL6 (not that rare in MZL), is BCL2 not rearranged, but positive /typical for MZL), it is IgM/IgD/MUM1/FOXP1/BCL2+ (typical for MZL). You may stain it for p53, mut BRAF, NOTCH1, kappa/lambda or perform NGS...     I think that there was a significant follicular colonization that caused confusion...
ugnius 2021-01-27 09:25	Thanx. p53+ heterogenous (accentuated on larger cells) up to 60% (not strong without front). Ig kappa slightly+ >> Ig lambda(-). NOTCH out of reach. BRAF in progress.     ADDITIONAL QUEST: if MZL- a large amount of large cells (blast excess)?
ugnius 2021-01-28 14:22	BRAF IH(-). Ig kapp faintly restricted. p53+ heterogenous. Added as last 3 photos (relapse)...
tzankov 2021-01-29 16:25	Thank you, Ugnius, agree - MZL with increased large cells, but not reaching criteria for transformation.
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