Table 2. Cases of Lymphoma with Optic Nerve Involvement and Central Nervous System Disease.
| Year/ Author |
Age/ Sex |
Diagnosis | Initial Visual Symptom |
Timing of visual Symptom |
Fundus Findings | Imaging | Lumbar Puncture |
Type of Biopsy / ON location |
Treatment | Outcome+ |
|---|---|---|---|---|---|---|---|---|---|---|
| B-cell lymphomas | ||||||||||
| Bullock, 1979 | 75 M | Poorly differentiated NHL | Decreased VA OD, Diplopia | 1 mo after chemotherapy initiation | normal | CT (-) Orbital xray (+) | 7 mononuclear cells, low glucose | ON at autopsy/ INT | XRT | Death, 31d |
| Guyer, 1990 | 57 F | Mixed cell NHL | Diplopia | 5 mo after diagnosis, 1 mos after CNS involvement | CRAO | CT (-) Carotid dopplar (-) | Atypical lymphocytes, increased intracranial pressure | ON at autopsy/ LPM | IT, XRT | Death, 8wk |
| Zaman, 1993 | 42 F | Centroblastic NHL | Decreased VA OU | 6 mo after chemotherapy | ON pallor, VF defect | CT (+), MRI (+) | wnl | No biopsy done | XRT | n/a |
| Saatci, 1999 | 14 M | Diffuse Large B-cell NHL | Decreased VA OS | 5 mo after chemotherapy | CRAO, CRVO | MRI (+) periventricular tumor infiltration | High protein | No biopsy done | IVc, IVs, IT XRT | Death, 24mo |
| Lee, 2002 | 56 M | Large B-cell NHL | Decreased VA OS, CN5 palsy | 4 mo after diagnosis | ON edema, RV engorgement, 4 quad IRH | MRI (+) | wnl | ON at autopsy/ LPM | IVs, XRT | Death, 3 weeks |
| El Kettani, 2006 | 75 M | Large B-cell NHL | Decreased VA OU, CN5 palsy | 8 years after diagnosis | B ON edema | CT (+) | Atypical Lymphocytes | No biopsy done | IVc, IVs, IT | Death, 2 mo |
| Zelefsky, 2008 | 72 F | Diffuse small B-cell | Decreased VA OD, VF defect | Presenting symptom | ON pallor | MRI (+) chiasm and tract | n/a | ON/ INT and LPM | IVc, PO | Death, 3mo |
| Matsuyama, 2013 | 84F | Diffuse large B-cell NHL | Decreased VA OU | Presenting symptom | n/a | MRI (+) suprasellar tumor | n/a | fronto-temporal craniotomy | PO | n/a |
| Matsuyama, 2013 | 67M | Malignant Lymphoma | Decreased VA OD | Presenting symptom | n/a | MRI (+) chiasm | n/a | fronto-temporal craniotomy | IVc | Death, 39mo |
| Case 2, 2014 | 25 F | Burkitt Lymphoma | Decreased VA OS | After 6 cycles of chemotherapy | Normal | MRI (+) Trigeminal nerve involvement | wnl | ON/ LPM | XRT | Hospice care, 2mo |
| T-cell lymphomas | ||||||||||
| Kitzmann, 2008 | 39 M | T-cell NHL | Decreased VA OU | After 2 cycles of chemotherapy | ON edema, flame hemorrhages, vein dilation, VF defects | MRI (+) | n/a | ON/ INT | IVc, XRT | Death, 3mo |
| Coleman, 2013 | 41M | Anaplastic Large T-cell NHL | Decreased VA OD, eye pain | Presenting symptom | n/a * asymmetric proptosis, ophthalmoplegia, chemosis | MRI (+) optic tract, chiasm, vasogenic edema of frontal lobe | n/a | total ethmoidectomy, maxillary antrostomy | IVc, XRT, PO | n/a |
M=male, F=female, NHL = Non Hodgkins lymphoma, OD=right eye, OS=left eye, OU=both eyes, CN=cranial nerve, VF= visual field, mo = months, CNS=central nervous system, CRAO = central retinal artery occlusion, CRVO = central retinal vein occlusion, ON = Optic Nerve, , n/a = information not available, (-) = negative findings, (+) = positive findings, wnl = within normal limits, INT = intrinsic, LPM = leptomeningeal, IVc = intravenous chemotherapy IVs = intravenous steroids, IT = intrathecal chemotherapy, PO = oral steroids, XRT = radiation therapy,
timing of death is with respect to onset of visual symptoms