Paraneoplastic Retinopathy

Paraneoplastic retinopathies are caused by antibodies produced in response to the presence of tumour antigens. These antibodies also react with retinal antigens, causing destruction of retinal tissue.

Two primary groups of paraneoplastic retinopathies exist. Cancer-associated retinopathy (CAR) is primarily associated with lung, breast and gynaecologic cancers and affects both rods and cones. Melanoma-associated retinopathy (MAR) affects only the rods and is associated with melanoma (metastatic, cutaneous or uveal).

CAR is associated with symptoms of photopsia, reduced visual acuity, colour vision impairment, photosensitivity, ring scotomas and nyctalopia.

MAR usually presents with a normal retinal appearance associated with reduced vision (6/30 or worse), nyctalopia and photopsias. A sub-set of this condition is vitelliform paraneoplastic retinopathy which has clinical signs essentially identical to acute exudative polymorphous vitelliform maculopathy (AEPVM). The two conditions are distinguished by a history of cancer.

Vitelliform paraneoplastic retinopathy (like AEPVM) is associated with small yellowish bleb-like lesions at the level of the RPE scattered along the vascular arcades. These lesions are also similar to the lesions of a vitelliform macular dystrophy and develop in a honeycomb-like pattern. Lesion patterns and progression are typically symmetrical between the two eyes.

Serous macular detachment with polymorphous subretinal yellowish deposits can occur with this condition and is associated with hyper-fluorescence on fundus autofluorescence. As the vitelliform material is reabsorbed, this hyper-fluorescence decreases.

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