Table 1.
Published cases of diffuse anterior retinoblastoma through 2012.
| Article author (Year) | Eye | Age/Sex | Presenting symptom | Eye exam |
US imaging | Diagnosis | Treatment | Retinal involvement under microscopy | Immuno-histochemical stains | Follow-up | |
|---|---|---|---|---|---|---|---|---|---|---|---|
| Anterior segment | Posterior segment | ||||||||||
| Garner et al. (1987) | OD | 7yo/ F | Redness, Blurring of vision | “Severe anterior uveitis with large iris nodules and cells and opacities in the anterior viteous” | – | – | Biopsy of iris, lens excision | Topical corticosteroids, oral prednisone 5 mg TID, sub-Tenon’s injection of methylprednisolone, lens excision, enucleation, orbital radiotherapy, adjuvant chemotherapy | 2 foci at extreme periphery of retina, minimal thickening, <1.5 mm in diameter | +NSE, −S100, −GFAP | Recurrent orbital retinoblastoma 8 mos after enucleation; No sign of further recurrence at 12 mos |
| Grossniklaus et al. (1998) | OD | 6yo/F | Unknown | Keratic precipitates, 4 + cells, IOP28 mmHg | Normal appearing retina, cells in inferior vitreous | Posterior vitreous detachment, no retinal abnormality | Anterior chamber fine needle aspiration biopsy | Topical prednisolone, dexamethasone 0.1%, betaxolol HCl 0.5%, Thiabendazole for 3 days, oral prednisone | Intraretinal tumor in peripheral retina | +NSE, + vimentin, -MAK-6, -AE 1,3, -GFAP, -S-100 protein | Unknown |
| 4 + cells, small “hypopyon”, IOP 34 mmHg | 1–2 + Vitreous cells | Topical prednisolone q1–2H, Dorzolamide HCl TID, Timolol maleate 0.25% qHS, Diclofenac sodium QID, dexamethasone qHS | |||||||||
| 4 + cells, dense “hypopyon” | 4 + vitreous cells | Rimexolone q1–2H, alpha clonidine 0.5% TID | |||||||||
| – | – | Enucleation | |||||||||
| Crosby et al. (2009) | OS | 9yo/ F | Blurry vision, redness, discoloration of iris | Pseudohypopyon, IOP 34 mmHg | “Possible small, inferior, peripheral mass in her left retina” | – | Anterior chamber fine needle aspiration biopsy | Enucleation | 3 × 1 mm tumor in peripheral inferior retina | Tumor Seeds: + TGF-β, + VEGF, -iNOS, -HIF1α | Unknown |
| Retina: + VEGF,-iNOS, -HIF1α | |||||||||||
| Longmuir et al. (2010) | OD | 8.5 yo/M | Unknown | 3–4 + cells, IOP 46 mmHg, “less prominent flare”, small hypopyon, multiple nodules on iris | Normal appearing retina | Iris root thickening to 1 mm for 360°, “mild anterior vitreous opacities” | Anterior chamber fine needle aspiration biopsy | Topical prednisolone 1% q1H, topical dorzolamide HCl, Timolol maleate, Brimonidine, Scopolamine HBr 0.25%, oral prednisone 30 mg PO q day, Enucleation, 6 cycles of vincristine, carboplatin, etoposide, external beam radiation (4140 cGy total) | No retinal involvement identified on 487 slides | +Synaptophysin , - Leukocyte common antigen, -CD34 | No recurrence at 5 years |
| Khetan et al. (2011) | OS | 3yo/F | “Unresolving anterior uveitis with secondary glaucoma” | Conjunctiva l congestion, pupil sluggishly reactive to light, “white, fluffy exudates”, IOP 31 mrnHs | Normal appearing retina | Normal | Anterior chamber fine needle aspiration biopsy | Topical prednisolone 1% q4H, Homatropine BID, Timolol maleate 0.5% + Brimondine acetate 0.2% BID, Enucleation | No retinal involvement identified | Unknown | No recurrence at time of publication |
| Herwig et al. (2011) | OS | 3yo/M | Discoloration of iris | Pseudohypopyon, IOP 26 mmHg | Normal appearing retina | No mass or calcification | Anterior chamber fine needle aspiration biopsy | Enucleation, 6 cycles of carboplatin, etoposide, vincristine | No retinal involvement identified | +Synaptophysin ,+NSE,-S-100 | No recurrence at 5 mos |