Clinical and immunologic characteristics of melanoma-associated retinopathy syndrome: Eleven new cases and a review of 51 previously published cases

Objective: To evaluate the signs, symptoms. and immune responses of patient s with melanoma-associated retinopathy (MAR) syndrome. Materials and Methods: We reviewed the clinical and immunologic findings of 62 MAR syndrome patients. They include 25 patients from our institution (1 1 not previously reported) and 37 patients reported from other institutions . Results: There were 33 men and seven women (no gender information is availa ble for the remaining 22 cases). Age at onset of the visual disturbance ave raged 57.5 years (range, 30-78). Visual acuity of 20/60 or better was initi ally present in 82%. Fundus examination was normal in 44%, optic disc pallo r was present in 23%. and retinal vessel attenuation was present in 30%. Vi treous cells were present in 30%. The latency from melanoma diagnosis to re cognition of MAR syndrome averaged 3.6 years (range, 2 months to 19 years). Seven patients sustained visual improvement with various treatment regimen s, especially with intravenous immunoglobulin and cytoreductive surgery (me tastasectomy). Indirect immunohistochemical staining of the bipolar layer w as typical, but several other retinal elements were also reactive. Tissue f rom a metastatic melanoma excised from one of the patients expressed antige ns that reacted with antiretinal antibodies. Conclusion: MAR syndrome demonstrates diverse clinical and immunologic feat ures. Treatment. especially intravenous immunoglobulin and cytoreductive su rgery (metastasectomy), improves vision in some cases.