Table 1

Characteristics and outcome for patients presenting with ocular adnexal lymphoma classified by histological type (REAL classification)

CharacteristicsHistological category of lymphoma
MZLLPLFCLDLCLH5All
NumberAll patients82/192 (43%)44/192 (23%)26/192 (14%)19/192 (10%)21/192 (11%)192
Solely adnexal  disease67/123 (54%)29/123 (24%)14/123 (11%)10/123 (8%)3/123 (2%)123/192 (64%)
Age at diagnosis (mean and range)All patients68 (18–87)63 (31–86)65 (18–86)73 (35–90)60 (3–81)66 (3–90)
Solely adnexal  disease68 (18–87)62 (31–86)61 (18–86)74 (39–85)58 (3–80)66 (3–87)
Sex (proportion men)All patients35/82 (43%)21/44 (48%)12/26 (46%)10/19 (53%)16/21 (76%)94/192 (49%)
Solely adnexal  disease29/67 (43%)15/29 (52%)6/14 (43%)5/10 (50%)2/3 (67%)57/123 (46%)
Clinical outcome characteristics in all patients
Systemic spread at diagnosisProportion15/82 (18%)15/44 (34%)11/26 (42%)5/19 (26%)16/21 (76%)62/192 (32%)
Odds ratio[1.0]2.3 (1.0, 5.3)3.3 (1.3, 8.5)1.6 (0.5, 5.1)14.3 (4.5, 45.1)
Extraorbital spread at diagnosisProportion15/82 (18%)15/44 (34%)12/26 (46%)9/19 (47%)18/21 (86%)69/192 (36%)
Odds ratio[1.0]2.3 (1.0, 5.3)3.8 (1.5, 9.9)4.0 (1.4, 11.6)26.8(6.9,102. 7)
Systemic spread at any timeProportion33/82 (40%)25/44 (57%)14/26 (54%)19/19 (47%)21/21 (100%)102/192 (53%)
Hazard ratio [1.0]1.0 (0.5, 2.0)1.7 (0.7, 3.9)2.0 (0.8, 5.1)4.1 (1.9, 8.9)
Extraorbital spread at any timeProportion33/82 (40%)25/44 (57%)16/26 (62%)13/19 (68%)21/21 (100%)108/192 (56%)
Hazard ratio[1.0]1.0 (0.5, 2.0)1.9 (0.8, 4.3)2.4 (1.0, 5.6)4.1 (1.9, 8.9)
All deathsProportion24/82 (29%)18/44 (41%)10/26 (39%)10/19 (53%)14/21 (67%)76/192 (40%)
Lymphoma related deathProportion11/82 (13%)12/44 (27%)8/26 (31%)7/19 (37%)11/21 (52%)49/192 (26%)
Hazard ratio[1.0]1.1 (0.5, 2.6)2.0 (0.8, 5.0)2.9 (1.1, 7.6)3.9 (1.7, 9.2)
Clinical outcome characteristics in patients with solely adnexal disease at diagnosis
Future extraProportion18/67 (27%)10/29 (35%)4/14 (29%)4/10 (40%)3/3 (100%)39/123 (32%)
Hazard ratio[1.0]0.9 (0.4, 1.9)1.3 (0.5, 4.0)1.9 (0.7, 5.8)3.1 (0.9, 10.5)
All deathsProportion19/67 (28%)10/29 (35%)4/14 (29%)5/10 (50%)1/3 (33%)39/123 (32%)
Lymphoma related deathsProportion6/67 (9%)4/29 (14%)3/14 (21%)3/10 (30%)1/3 (33%)17/123 (14%)
Hazard ratio[1.0]0.5 (0.1, 2.3)2.1 (0.5, 8.7)2.5 (0.6, 11.2)3.2 (0.4, 26.9)
  • Lymphoma types: MZL = marginal zone lymphoma, LPL = diffuse lymphoplasmacytoid/lymphoplasmacytic lymphoma, FCL = follicle centre lymphoma, DLCL = diffuse large B cell lymphoma, H5 = rare histologic variants (see Table 2).

  • Lower and upper 95% confidence limits are given in parentheses after odds and hazard ratios. [1.0] is the reference datum.

  • “Systemic spread” refers to remote non-contiguous disease. “Extraorbital spread” refers to both remote disease and local extraorbital extension.

  • These data refer to patients presenting without a previous history of lymphoma or without extraorbital lymphoma diagnosed (usually as a result of staging) within 3 months of biopsy.