Paraneoplastic Vitelliform Retinopathy: Clinicopathologic Correlation and Review of the Literature

Abstract

Traditionally the paraneoplastic retinopathies have been classified into two groups: melanoma-associated retinopathy (MAR) and cancer-associated retinopathy (CAR). MAR occurs in individuals with metastatic cutaneous or uveal melanoma and is characterized by nyctalopia, photopsias, and variable vision loss. In most cases, the fundus is essentially normal in appearance. More recently, there have been multiple reports of a MAR-like retinopathy with associated detachments of the retinal pigment epithelium (RPE) and neurosensory retina. Such a clinical presentation has been termed paraneoplastic vitelliform retinopathy. In the present report, we describe an 80-year-old male with metastatic cutaneous melanoma who developed paraneoplastic vitelliform retinopathy. For the first time, histopathology from enucleation specimens provides a clinicopathologic disease correlation with focal abnormalities in the inner nuclear and outer plexiform layers.

MEK inhibitor-associated retinopathy (MEKAR) in metastatic melanoma: Long-term ophthalmic effects

“…The term MEKAR is proposed in analogy to CAR, cancer associated retinopathy or MAR, melanoma associated retinopathy, both rare eye conditions with severe functional deficits observed in metastatic disease [23,24]. Auto-immunologic based cross reactions between tumour antigens and retinal tissues were described.…”

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

“…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.…”

Paraneoplastic Vitelliform Retinopathy Secondary to Metastatic Melanoma

“…Recently, a vitelliform retinopathy has been described in both metastatic cancer and melanoma patients: in 2001, Borkowksy reported the first patient as a case of MAR with unusual retinal features [1]. Since then, only 20 cases have been reported [2][3][4][5]. This auto-immune disorder is caused by circulating autoantibodies, cross-reacting with the neuroretina and retinal pigment epithelium (RPE) [2].…”

“…Since then, only 20 cases have been reported [2][3][4][5]. This auto-immune disorder is caused by circulating autoantibodies, cross-reacting with the neuroretina and retinal pigment epithelium (RPE) [2].…”