Paraneoplastic retinopathy with multiple detachments of the neurosensory retina and autoantibodies against interphotoreceptor retinoid binding protein (IRBP) in cutaneous melanoma

Abstract

A 74-year-old Caucasian man developed gradual bilateral visual loss over 3 months. He had bilateral chronic open-angle glaucoma treated with trabeculectomy. Past medical history included cutaneous melanoma of the face and neck excised 14 months earlier. The patient then developed brain metastasis treated with external beam radiotherapy 6 months prior and liver, axillary lymph nodes and lung metastasis treated with systemic chemotherapy 3 months earlier.On ocular examination, best corrected visual acuities were 20/70 in the right eye (OD) and 20/60 in the left eye (OS).

Paraneoplastic and non-paraneoplastic retinopathy and optic neuropathy: Evaluation and management

“…2), often accompanied by overlying serous macular detachment, with a tendency of the vitelliform material to gravitate inferiorly and layer out within the areas of subretinal fluid. 14,33,69,93,120,122,136,147,148,200,214,265,271 Involvement is typically bilateral, and although most cases associated with choroidal melanoma were in monocular patients, two treated with plaque brachytherapy alone had bilateral fundus lesions consistent with PVM. 14,B FA typically demonstrates blockage of background choroidal fluorescence by the vitelliform lesions with late staining, 14,20,93,122,136,148,200,265 although in some cases the lesions displayed a mild early hyperfluorescence.…”

“…To our knowledge, 23 cases of PVM, albeit under different designations, have been reported in the literature (Table 1). 14,20,33,35,69,93,120,122,136,147,148,200,214,265,271,301 All but two carried a known diagnosis of malignancy. Until recently, all known cases were associated with either choroidal or cutaneous melanomas, leading many authors to speculate these may be atypical variants of MAR; however, three recent reports demonstrated similar findings in patients with non-melanoma malignancies.…”

“…The average age of onset is 59 years (range, 33e80 years), with an equal sex distribution. 14,20,33,35,69,93,120,122,136,147,148,200,214,265,271,301 The most common presentation is nyctalopia, although some patients experienced blurry vision, metamorphopsia, shimmering photopsias, glare, and halos. Clinically, PVM may more closely resemble other vitelliform retinopathies, such as acute exudative polymorphous vitelliform maculopathy, or the multifocal variant of Best's disease, than MAR or CAR.…”

“…14,93 The absence of focal choroidal thickening on OCT also helps exclude choroidal metastases. Serological testing has identified autoantibodies directed against bipolar cells, 35,214,301 rod outer segment protein (ROS, 120-kDa), 265 peroxiredoxin 3 (PRDX3, 26-kDa), 147 bestropin-1 (68-kDa), 69 carbonic anhydrase II (CAII, 30-kDa), 14,20 interphotoreceptor retinoid binding protein (IRBP, 145-kDa), 33 and unnamed 35-kDa 20 and 68-kDa 14 proteins. Meanwhile, ERG and electrooculography (EOG) evaluation has yielded variable results.…”

Paraneoplastic Vitelliform Retinopathy: Clinicopathologic Correlation and Review of the Literature

“…5,6,8,9,12,14,16,18 In 2001 Borkowski et al described 2 cases of a MAR-like syndrome with unusual fundus features. 6 In the first case, there were oval, white lesions at the level of the RPE, and in a second, there were scattered, well-circumscribed, atrophic lesions in the posterior pole and mid-periphery.…”