title: Aggressive metastasising adenocarcinoma of the retinal pigment epithelium with trisomy 21.
abstract: 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.
authors.affiliation: Department of Ophthalmology and Eye Hospital, University Erlangen-Nürnberg, Schwabachanlage 6, 91054 Erlangen, Germany. ludwig.heindl@augen.imed.uni-erlangen.de
authors.forename: L M
authors.initials: LM
authors.lastname: Heindl
authors.forename: G O H
authors.initials: GO
authors.lastname: Naumann
authors.forename: F E
authors.initials: FE
authors.lastname: Kruse
authors.forename: L M
authors.initials: LM
authors.lastname: Holbach
mesh_terms: D000230:Adenocarcinoma / Q000473:pathology / Q000556:secondary*; D000328:Adult; D001932:Brain Neoplasms / Q000556:secondary; D004314:Down Syndrome; D005123:Eye / Q000473:pathology; D017809:Fatal Outcome; D006801:Humans; D008297:Male; D009361:Neoplasm Invasiveness; D010857:Pigment Epithelium of Eye; D019572:Retinal Neoplasms / Q000473:pathology*; D013120:Spinal Cord Neoplasms / Q000556:secondary