1Bölöni R., 2Dirsch O., 3Krüger C., 1Schüler A., 1Bornfeld N.

1Universitäts-Augenklinik und 2Institut für Pathologie, Hufelandstr. 55, 45122 Essen; 3Universitätsklinik für Gesichts- und Kieferchirurgie, Henricistr. 92, 45136 Essen

Introduction: Schwannomas are rare benign peripheral nerve sheath neoplasms of Schwann cell origin. They have been reported in soft tissues, peripheral nerves and in the orbit, but rarely intraocular. In these cases they can be misdiagnosed as amelanotic uveal melanoma.
Case report: A 20-year-old woman presented with a 6 months' history of gradual deterioration of vision in the right eye. On examination the vision was reduced to 1/35, the intraocular pressure was normal. Slit lamp examination showed normal anterior segments. There was no proptosis bulbi. Ophthalmoscopy revealed a non pigmented tumor on the posterior pole with optic disc oedema and tortuosity of retinal vessels with focal exsudation. Due to the clinical presentation, the ultrasonography and the MR image the diagnosis of an intraocular melanoma with extraocular extension was supposed. Glioma of the optic nerve, metastasis of a primary tumor and lymphoma were considered as differential diagnosis. Transscleral biopsy could not deliver a definitive diagnosis. After removing the tumor mass histological examination showed typical features of a benign schwannoma.
Discussion: Although extremely rare, schwannomas with intraocular extension must be considered in the differential diagnosis of non-pigmented uveal melanomas. Thus the patients can undergo an adequate therapy consisting of local excision.

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