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Abstract #56541 Published in IGR 16-1

Choroidal schwannoma in a 6-month-old girl

Chen JJ; Kamberos NL; O'Dorisio MS; Syed NA; Boldt C
Journal of AAPOS 2014; 18: 197-199


Choroidal schwannomas are exceedingly rare in children, with only 6 cases reported in children younger than 18 years of age and none in those younger than 9 years. We report a 6-month-old infant presenting with a large noncalcified amelanotic mass with secondary glaucoma that mimicked an atypical retinoblastoma, leading to emergent enucleation for therapeutic and diagnostic purposes. Pathology revealed a total retinal detachment, glaucomatous damage, and a large mass arising from the peripapillary posterior choroid with areas of Antoni A pattern and S-100 staining consistent with the diagnosis of an intraocular schwannoma. This is the first intraocular schwannoma described in an infant.

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Classification:

9.4.8 Glaucomas associated with intraocular tumors (Part of: 9 Clinical forms of glaucomas > 9.4 Glaucomas associated with other ocular and systemic disorders)
9.1.1 Congenital glaucoma, Buphthalmos (Part of: 9 Clinical forms of glaucomas > 9.1 Developmental glaucomas)
2.12 Choroid, peripapillary choroid, peripapillary atrophy (Part of: 2 Anatomical structures in glaucoma)



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