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Aggressive metastasising adenocarcinoma of the retinal pigment epithelium with trisomy 21.

Heindl LM, Naumann GO, Kruse FE, Holbach LM

Department of Ophthalmology and Eye Hospital, University Erlangen-Nürnberg, Schwabachanlage 6, 91054 Erlangen, Germany. ludwig.heindl@augen.imed.uni-erlangen.de

This case report describes a 37-year-old man with a blind eye with opaque media. Histopathology revealed an aggressive adenocarcinoma of the retinal pigment epithelium with marked invasion of the choroid, retina, sclera and--not previously reported--the orbital and cranial optic nerve and subarachnoid space. The tumour seeded into the lumbar spinal-cord space. Metastatic foci--not in continuity with the primary process--developed in the parietal lobe and cerebellopontine angle. Adenocarcinoma of the retinal pigment epithelium, which occurs rarely in longstanding blind eyes, may exhibit aggressive behaviour with life-threatening risk of metastatic spread.

Published 27 February 2008 in Br J Ophthalmol, 92(3): 389-91.
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