Schwannoma is a proliferation of neoplastic Schwann cells. Whereas schwannomas of the head and neck region are common, intraocular tissues are rarely affected. Uveal schwannoma has been aptly called a "pseudomelanoma", reflecting the difficulty in its clinical distinction from uveal malignant melanoma. Most of our current knowledge on intraocular schwannoma is limited to case reports, short case series, and non-comprehensive literature reviews. Three isolated reports of uveal schwannoma with extrascleral extension exist in literature, but the prognostic significance of this growth pattern is unknown. We present a patient with choroidal schwannoma with extrascleral extension and review 46 previously reported cases of uveal schwannomas to delineate clinical and pathologic characteristics of these intraocular tumors with a specific emphasis on schwannoma with extraocular extension.
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