Table 1 Literature review of EMZL since 1994
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 | Â | 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 | Â | 45/M | NA/LP | Y | Uvea | CD79a+, CD20+, BSAP+, MUM1+, CD38+, CD138+, Vs38c+, Kappa LC-, Lambda LC+, IgM+, IgD- | Enucleation, Radiotherapy | N | 120 |
6 | Â | 64/M | NA/LP | Y | Uvea | CD79a+, CD20+, BSAP+, MUM1+, CD38-, CD138+, Vs38c+, Kappa LC-, Lambda LC+, IgM+, IgD- | Enucleation | N | 108 |
7 | Â | 33/M | NA/CF | Y | Uvea | CD79a+, CD20+, BSAP+, MUM1+, CD38+, CD138-, Vs38c+, Kappa LC-, Lambda LC+, IgM+, IgD- | Enucleation | N | 84 |
8 | Â | 63/M | NA/0.05 | Y | Uvea | CD79a+, CD20+, BSAP+, MUM1+, CD38+, CD138-, Vs38c+, Kappa LC+, Lambda LC-, IgM+, IgD- | Enucleation | N | 60 |
9 | Â | 59/F | NA/LP | Y | Uvea | CD79a+, CD20+, BSAP+, MUM1+, CD38+, CD138-, Vs38c-, Kappa LC-, Lambda LC-, IgM+, IgD- | Enucleation | N | Unknown |
10 | Â | 66/M | NA/LP | Y | Uvea | CD79a+, CD20+, BSAP+, MUM1+, CD38+, CD138-, Vs38c-, Kappa LC-, Lambda LC+, IgM+, IgD- | Enucleation | N | Unknown |
11 | Â | 73/M | NA/LP | Y | Uvea | CD79a+, CD20+, BSAP+, MUM1+, CD38+, CD138+, Vs38c-, Kappa LC+, Lambda LC-, IgM+, IgD- | Enucleation | Y | 2 |
12 | Â | 81/M | NA/LP | Y | Uvea | CD79a+, CD20+, BSAP+, MUM1+, CD38+, CD138+, Vs38c-, Kappa LC-, Lambda LC+, IgM+, IgD- | Enucleation | Y | 180 |
13 | Â | 73/F | NA/NLP | Y | Uvea | CD79a+, CD20+, BSAP+, MUM1+, CD38+, CD138+, Vs38c+, Kappa LC+, Lambda LC-, IgM+, IgD- | Enucleation | N | Unknown |
14 | Â | 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 | Â | 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 | Â | 46/M | NA/0.2 | N | Choroid | CD20+++, CD79a+++, CD3+ | Chemotherapy | N | 84 |
26 | Â | 63/M | NA/0.16 | Conjunctiva | Choroid | CD20+++, CD5+, CD10-, Bcl6-, CyclinD1-, Ki67 50% | Radiotherapy Chemotherapy | N | 60 |
27 | Â | 54/F | NA/0.625 | N | Choroid | CD20+++, CD5+, CD23-, Bcl6-, CyclinD1-, Kappa LC+, Lambda LC+++ | Radiotherapy | N | 144 |
28 | Â | 64/F | NA/0.005 | Orbit | Choroid | CD20+++, CD5+, CD10-, CD138+, Bcl6-, CyclinD1-, Kappa LC+, Lambda LC+++, Ki67 5% | Radiotherapy | N | 12 |
29 | Â | 54/F | NA/0.5 | Orbit | Choroid | CD20+, CD5+, CD3+, kappa LC-, Lambda LC- | Radiotherapy Chemotherapy | N | 120 |
30 | Â | 40/M | NA/0.8 | N | Choroid | CD20+, Bcl2-, Kappa LC+ | Radiotherapy Chemotherapy | N | 156 |
31 | Â | 53/F | NA/0.1 | Lacrymal gland | Choroid | CD20++, CD3+, CD5+, CD8+, Bcl2+++, Bcl6++, Kappa LC+++, Lambda LC+, Ki67- | Chemotherapy | N | 53 |
32 | Â | 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 | Â | 71/M | R/0.05 | Conjunctiva | Iris, choroid | NA | Radiotherapy | N | 36 |
36 | Â | 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 |