Melanoma-associated paraneoplastic retinopathy: case report and review of the literature

Abstract

A 51-year-old white male suffering from metastatic malignant melanoma of the skin presented with lymph node metastases and paraneoplastic retinopathy 4 years after resection of the primary tumour. There were no cerebral metastases. Ocular symptoms consisting of night blindness and sensations of 'shimmering lights' persisted after total resection of the inguinal lymph node metastases and administration of dacarbazine and prednisone. Perimetry of both eyes was abnormal with concentric restriction. Electroretinography showed significantly reduced amplitudes in both eyes. Only 11 patients with melanoma-associated retinopathy (MAR) have been described. High titres of autoantibodies against whole retina extract were detected by enzyme-linked immunosorbent assay (ELISA) reactions. Indirect immunohistochemistry showed strong autoantibody activity against retinal bipolar cells.

Cancer‐retina antigens as potential paraneoplastic antigens in melanoma‐associated retinopathy

“…16 Moreover, the difference in the level of this dysfunction could explain why retinal degenerations are not always found in both eyes of MAR patients. 10,12 Using our mouse melanoma model, we also found that only 62% of affected animals had retinopathy in both eyes.…”

“…We found that melanoma-bearing mice showed similar signs of retina degeneration as reported for MAR patients, including the reduction of aand b-waves, degeneration of photoreceptor cells, bipolar cells and pigment epithelium. [8][9][10][11][12][13] Since this retina degeneration in tumor-bearing mice appeared to be a paraneoplastic event, which was independent from any ocular tumor invasion and from overexpression of the ret transgene, we suggested that ret transgenic mice could be a good animal model of MAR.…”

“…[3][4][5][6][7] Melanoma-associated retinopathy (MAR) is a rare paraneoplastic syndrome in patients with metastatic melanoma characterized by visual disorders and the presence of serum AA against retinal proteins. [8][9][10][11][12][13][14] MAR is closely related to cancer-associated retinopathy, 15 which often occurs in lung cancer patients but differs in ophthalmological symptoms and probably in the antigens recognized, which are unknown today (for review see Ref. 16).…”

“…[8][9][10][11] Sera from cancer-associated retinopathy patients stain strongly photoreceptor cells but only weakly bipolar cells, while sera from MAR patients strongly react with bipolar cells [8][9][10][11] suggesting that the MAR syndrome is induced by the degeneration of bipolar cells. Indeed, the histological examination of the retina from MAR patients indicates the degeneration of second-order neurons-bipolar cells.…”

Cutaneous Ulceration after Injection of Polyethylene-Glycol-Modified Interferon Alpha Associated with Visual Disturbances in a Melanoma Patient

“…blurred vision. The question was whether the patient had melanoma-associated retinopathy [31], an early interferon-associated retinopathy or an anterior ischemic optic neuropathy after interferon treatment. A melanoma-associated retinopathy was excluded by a conventional electroretinogram of the rods and cones and by a normal subjective night vision.…”