Intraocular Tumors – Melanocytic
Differential Diagnosis of iris nodules
- Cyst, Epithelial invasion
- serous or solid cysts following surgery
- Retained foreign body -usually secondary pigmentation of iris → chronic iridocyclitis and PAS
- Fungal endophthalmitis -Irregular yellow-white mass, cell in AC, +/-hypopyon
- Iridocyclitis -Granulomatous nodules –superficial or deep–associated with
- Koeppe nodules located at the pupillary border
- Busacca nodules located on anterior iris surface
- Iris freckle -stationary, light-dark, flat, anterior, with increased pigment, but no hyperplasia
- Iris Nevus -discretemass on anterior iris surface
- Composed of benign nevus cells.
- Increased incidence in neurofibromatosis-1
- Cogan-Reese iris nevus syndrome
- Acquired diffuse nevus: associated with
- ICE syndrome
- Iris pigment epithelial cysts -encompass both layers of iris and produce localized stromal elevation. May need B-scan or trans-illumination to see.
- Iris pigment epithelial proliferation results from congenital or acquired (trauma/surgery) –composed of plaques of pigment epithelium –black, velvety color.
- Juvenile xanthogranuloma -yellow/grey iris lesions with orange skin
- Lesions in 1st yearof life.
- Assoc. 1. with spontaneous hyphema
- Secondary glaucoma
- Diffuse granulomatous reaction with lipid filled histiocytes and touton giant cells Regress spontaneously
- localized or diffuse, pedunculated or flat
- Electron microscopy required to differentiate from amelanotic spindle cell melanoma
- Leukemia -very rare
- Nodularor diffuse milky lesions with intense hyperemia.
- Iris loses architecture and becomes thickened.
- Lisch Nodules
- one of the diagnostic criteria for NF-1
- Multiple flat or raised tan to brown lesions
- Composed of collections of nevus cells
- Malignant melanoma
- Nodular of flat –usually peripheral
- 80% inferior + inferotemporal
- May have nutrient vessel, satellite pigmentation
- Pupil may dilate irregularly
- Unilateral, heterochromia irides
- May have oculodermal form with eyelid + brow involved
- Has malignant potential
- White foci on iris surface or in angle
- May have pseudohypopyon
- Retinoblastoma present in posterior chamber
- Tapioca Melanoma
- Often associated with unilateral glaucoma.
- Tapioca-like nodules over part or all of iris
- Metastatic Carcinoma
- Gelatinous to white vascularized nodules on iris surface.
- May be associated with anterior uveitis, glaucoma, rubeosis, and hyphema.
|Nervus||<5mm diameter||<2mm thick|
|Group I (very favorable)||solitary tumor <4DD||multiple tumors|
|III||at equator or anterior||>10DD posterior|
|IV||multiple tumors, some >10DD||anteriorto ora serrata|
|V(least favorable)||massive tumor > ½ retina||vitreous seeding|
|A||Small(<3mm)||>3mm from fovea||>1.5mm from optic nerve|
|B||(>3mm)||confined to retina|
|C||Localizedvitreous or subretinal seeding.||<6mm from tumor|
|D||Diffuse vitreous or subretinal seeding.||>6mm from primary|
|E||No visual potential or tumor in anteriorsegment, in or onciliary body, neovascular glaucoma,vitreous hemorrhage, phthisical eye, orbital extension/proptosis.|
|Lung 40%||Breast 68%|
|Unknown 29%||Lung 12%|
|GI 9%||Unknown 12%|
|Kidney 6%||Others 4%|
|Prostate 6%||GI 2%|
|Skin 4%||Skin 1%|