First reported by Gass in 1973, these lesions can be mistaken for choroidal melanoma or retinoblastoma. The majority are picked up in children median age 7.5, (range 0.4-60) presenting with a squint or reduced vision.

Characteristics include:-

i) slightly elevated charcoal grey mass
ii) extending in a fanlike way towards the periphery
iii) blending with surrounding RPE with no surrounding RPE/choroidal atrophy, retinal exudation, haemorrhage or detachment and no vitreous inflammation
iv) contraction of inner retinal surface
v) covered by thick grey retinal and preretinal tissue.

They may occur in the peripapillary area, macula or periphery (hence the late presentations) and may occur alone or in association with other conditions such as neurofibromatosis.  Bilateral cases have been reported.  Ophthalmology Dec 2008