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Table 1 Literature review of EMZL since 1994

From: Primary extranodal marginal zone B-cell lymphoma with diffuse uveal involvement and focal infiltration of the trabecular meshwork: a case report and review of literature

No. of cases Ref/Year Age/Gender Eye/Initial BCVA Extraocular extension Site Pathology findings Treatment Systemic work-up Follow-up (months)
1 5/2000 51/F L/0.5 N Iris CD20+, CD19+, Kappa LC+ Radiotherapy Enucleation N 72
2 12/2002 63/M NA/NLP N Uvea CD79a+, CD20+, BSAP+, MUM1+, CD38+, CD138-, Vs38c+, Kappa LC-, Lambda LC+, IgM-, IgD- Enucleation N 156
3   40/F NA/NLP Y Uvea CD79a+, CD20+, BSAP+, MUM1+, CD38+, CD138-, Vs38c-, Kappa LC-, Lambda LC+, IgM+, IgD- Enucleation, Radiotherapy Y 300
4   40/M NA/HM Y Uvea CD79a+, CD20+, BSAP+, MUM1+, CD38+, CD138±, Vs38c+, Kappa LC+, Lambda LC-, IgM+, IgD- Enucleation, Radiotherapy N 216
5   45/M NA/LP Y Uvea CD79a+, CD20+, BSAP+, MUM1+, CD38+, CD138+, Vs38c+, Kappa LC-, Lambda LC+, IgM+, IgD- Enucleation, Radiotherapy N 120
6   64/M NA/LP Y Uvea CD79a+, CD20+, BSAP+, MUM1+, CD38-, CD138+, Vs38c+, Kappa LC-, Lambda LC+, IgM+, IgD- Enucleation N 108
7   33/M NA/CF Y Uvea CD79a+, CD20+, BSAP+, MUM1+, CD38+, CD138-, Vs38c+, Kappa LC-, Lambda LC+, IgM+, IgD- Enucleation N 84
8   63/M NA/0.05 Y Uvea CD79a+, CD20+, BSAP+, MUM1+, CD38+, CD138-, Vs38c+, Kappa LC+, Lambda LC-, IgM+, IgD- Enucleation N 60
9   59/F NA/LP Y Uvea CD79a+, CD20+, BSAP+, MUM1+, CD38+, CD138-, Vs38c-, Kappa LC-, Lambda LC-, IgM+, IgD- Enucleation N Unknown
10   66/M NA/LP Y Uvea CD79a+, CD20+, BSAP+, MUM1+, CD38+, CD138-, Vs38c-, Kappa LC-, Lambda LC+, IgM+, IgD- Enucleation N Unknown
11   73/M NA/LP Y Uvea CD79a+, CD20+, BSAP+, MUM1+, CD38+, CD138+, Vs38c-, Kappa LC+, Lambda LC-, IgM+, IgD- Enucleation Y 2
12   81/M NA/LP Y Uvea CD79a+, CD20+, BSAP+, MUM1+, CD38+, CD138+, Vs38c-, Kappa LC-, Lambda LC+, IgM+, IgD- Enucleation Y 180
13   73/F NA/NLP Y Uvea CD79a+, CD20+, BSAP+, MUM1+, CD38+, CD138+, Vs38c+, Kappa LC+, Lambda LC-, IgM+, IgD- Enucleation N Unknown
14   63/M NA/N LP N Uvea CD79a+, CD20+, BSAP+, MUM1+, CD38+, CD138+, Vs38c+, Kappa LC+, Lambda LC-, IgM+, IgD- Enucleation N 120
15 6/2003 83/F L/0.2 N Iris CD20/l-26+, CD79a+, CD3-, CD45RO/UCHL-1- Radiotherapy N 8
16 13/2005 45/M L/0.16 N Choroid CD79a+, CD20+, BCL2+, CD43+, CD3-, CD5-, CD23-, CyclinD1-, Kappa LC+, IgM+, Ki67 10% Radiotherapy N 17
17 7/2008 84/F R/0.63 N Ciliary body CD79a+, CD20+, CD43±, IgM+, CD5-, CD23-, CyclinD1-, Ki-67 5%-15% Radiotherapy N 4
18 14/2008 57/F L/0.5 Conjunctiva Choroid CD20+, Bcl2+, CD5-, CD23-, CD43-, Bcl6-, CyclinD1-, Kappa LC+ Radiotherapy N 6
19   85/M NA N Choroid CD20+, CD19+, CD5-, Lambda LC+ Radiotherapy N 3
20 15/2009 68/M L/HM Conjunctiva Choroid CD20+, CD3±, Lambda LC+, kappa LC- Radiotherapy N 60
21 16/2010 80/M R/0.1 Epibulbar tumor Iris, ciliary body, choroid CD3-, CD5-, CD10-, CD20+, CD23-, CD38-, CD43±, CD56-, CD57-, CD79a+, CD138-, Bcl-2+, Bcl-6-, CyclinD1-, IgM-, Kappa LC-, Lambda LC-, Ki-67 < 10% Enucleation N 28
22 17/2011 49/M R/CF Orbit, sphenoid bone Choroid CD20+, CD3-, CD5-, CD10- Chemotherapy Radiotherapy N NA
23 18/2012 62/M L/0.67 Orbit, optic nerve Choroid CD79+, CD20+, BCL2+, CD10+ (focally), CyclinD1- Radiotherapy N 6
24 19/2013 73/F NA/0.625 Conjunctiva, orbit Choroid CD20+++, CD5+, Bcl6+, CyclinD1+, Kappa LC+, Lambda LC -, Ki67 5-10% Radiotherapy N 6
25   46/M NA/0.2 N Choroid CD20+++, CD79a+++, CD3+ Chemotherapy N 84
26   63/M NA/0.16 Conjunctiva Choroid CD20+++, CD5+, CD10-, Bcl6-, CyclinD1-, Ki67 50% Radiotherapy Chemotherapy N 60
27   54/F NA/0.625 N Choroid CD20+++, CD5+, CD23-, Bcl6-, CyclinD1-, Kappa LC+, Lambda LC+++ Radiotherapy N 144
28   64/F NA/0.005 Orbit Choroid CD20+++, CD5+, CD10-, CD138+, Bcl6-, CyclinD1-, Kappa LC+, Lambda LC+++, Ki67 5% Radiotherapy N 12
29   54/F NA/0.5 Orbit Choroid CD20+, CD5+, CD3+, kappa LC-, Lambda LC- Radiotherapy Chemotherapy N 120
30   40/M NA/0.8 N Choroid CD20+, Bcl2-, Kappa LC+ Radiotherapy Chemotherapy N 156
31   53/F NA/0.1 Lacrymal gland Choroid CD20++, CD3+, CD5+, CD8+, Bcl2+++, Bcl6++, Kappa LC+++, Lambda LC+, Ki67- Chemotherapy N 53
32   57/M NA/0.32 Conjunctiva Choroid CD20+++, CD5+, CD23-, Bcl6+, Kappa LC-, Lambda LC-, Ki67 20% Chemotherapy N 33
33 20/2013 71/M L/0.2 Conjunctiva, optic nerve Choroid CD20+, CD43+, Bcl2+, CD3- Chemotherapy N 12
34 21/2013 71 /F L/0.5 Conjunctiva Iris, choroid NA Radiotherapy N 44
35   71/M R/0.05 Conjunctiva Iris, choroid NA Radiotherapy N 36
36   75/M R/0.5 Conjunctiva, orbit Iris, ciliary body, choroid NA Radiotherapy Unknown 0
37-52 22/2014 NA NA NA Choroid NA NA NA NA
53-60 23/2014 NA NA NA NA NA NA NA NA
61 Present case 38/M R/0.1 Orbit, optic nerve Iris, ciliary body, choroid, trabecular meshwork CD5+, CD10-, CD20+, CD23+, CD38+, CD79a+, Bcl-2+, Bcl-6-, CyclinD1-, Kappa LC+, Lambda LC+, Ki-67 5%-10% Enucleation N 18
  1. BCVA, best corrected visual acuity; CF, counting figure; F, female; HM, hand motion; L, left; LP, light perception; M, male; N, no; NA, not available; NLP, no light perception; R, right; Y, yes.