Melanoma-associated retinopathy H35.-

Melanoma-associated retinopathy (MAR) is a paraneoplastic autoimmune manifestation in malignant melanoma characterized by sudden acquired night blindness with light sensations.

Melanoma-associated retinopathy (MAR), like carcinoma-associated retinopathy (CAR), is a tumor-associated retinal disease. In contrast to CAR, the immune response in MAR is not directed against antigens of photoreceptors and ganglion cells, but against retinal bipolar cells, which are predominantly located downstream of the rods. This may be caused by cross-reacting antibodies against melanoma antigens, which also recognize retinal proteins, or by retinal proteins ectopically expressed in melanoma.

The type and progression of emerging retinopathies can be quantified by optical coherence tomography (OCT) and, in the case of pronounced findings, by fluorescein angiography (FLA).

Antibody responses have been described in the literature primarily against retinal bipolar cells, but antigens outside the bipolar cell layer are increasingly associated with melanoma-associated retinopathy. Melanoma patients with MAR are thought to have a better prognosis due to the autoimmune reaction.

A 51-year-old hitherto ophthalmologically healthy patient with malignant skin melanoma and sudden onset of nyctalopia is presented. In addition to a decrease in visual acuity, a concentric visual field loss, a marked decrease in sensitivity of the rod and cone apparatus in adaptometry, significantly reduced b-waves with preserved a-waves in the flash ERG were registered in both eyes with otherwise unremarkable morphologic findings. Increased serum titers of antibodies (IgG) against retinal bipolar cells were further measured.

1. Groselli S et al (2021) MEK inhibitor-associated retinopathy during binimetinib therapy for cutaneous malignant melanoma. Ophthalmologist 118:169-174.
2. Hartmann TB et al. Novel tumor antigens associated with melanoma-associated retinopathy. Act Dermatol 30 - P52