Cancer-associated retinopathy preceding the diagnosis of cancer

Abstract

BackgroundThe early diagnosis of cancer is of crucial importance and a key prognostic factor. Cancer-associated retinopathy (CAR) can be symptomatic prior to other manifestations directly related to malignant tumors. The aim of this study was to show that, in selected cases, ophthalmic findings are consistent enough with the diagnosis of CAR to trigger investigations aimed at detecting a previously unknown malignancy.MethodsThis was a monocentric retrospective case series performed in a tertiary referral center. Patients with a diagnosis of CAR were included. Diagnosis was based on the clinical presentation, the visual field and electroretinogram alterations. The clinical presentation, visual field testing and electroretinographic results were analyzed as well as the malignancies identified following the diagnosis of CAR. Follow-up data was collected.ResultsFour patients (two men, two women, median age 65.5 years) were included. All patients presented with posterior segment inflammation at initial presentation as well as advanced visual field loss and an extinguished electroretinogram. The best corrected decimal visual acuity was 0.8 or better in both eyes of three patients and decreased to 0.3 OD and O.2 OS in one patient due to a bilateral macular edema. No patient had a previously known history of cancer. Once the diagnosis of CAR was made, investigations aimed at identifying a malignant tumors subsequently led to the diagnosis of two cases of small cell lung tumors, of one prostate carcinoma and of a uterine sarcoma. The treatment of CAR included plasmapheresis, systemic corticosteroids, azathioprine, cyclosporine and periocular or intraocular corticosteroid injections. In all cases the intraocular inflammation resolved, but pigment mottling, diffuse retinal atrophy, optic disc pallor and arterial narrowing were among manifestations observed during the follow-up of the patients.ConclusionIn selected patients, findings suggestive of CAR can be useful for the early detection of a cancer.Electronic supplementary materialThe online version of this article (10.1186/s12886-018-0948-2) contains supplementary material, which is available to authorized users.

Progressive visual loss and severe retinal degeneration in a captive Kodiak bear (Ursus arctos mittendorfi)

“…Diagnosis of CAR in humans is often based on a varying degree of attributable clinical signs and diagnostic findings, including photosensitivity, attenuated retinal arterioles viewed via fluorescein angiography, vision loss with a ring scotoma, extinguished ERG, optic disc pallor, retinal vasculitis, fundic hyper‐reflectivity and intraocular inflammation. CAR is only confirmed when a tumour is identified within the patient and no other discernible causes for the pathologies can be identified 32 45–50. Western blot serology can be performed to identify circulating retinal autoantibodies in humans; however, the technique has poor specificity (as low as 55.6 per cent) 32 47 51.…”

Cancer-Associated Retinopathy due to Clear Cell Renal Carcinoma

“…The ERG abnormality was subtle and not symmetric where the LE’s electronegative waveform suggested a post-phototransduction defect that can be seen with the AIRs and CAR. Although this patient did not have classic systemic findings associated with paraneoplastic syndromes, such as anemia, fever, cachexia, or hypercalcemia, any patient with AIR should undergo a malignancy workup [1]. In particular, our patient’s age raised our level of suspicion for CAR and prompted the imaging that led to uncovering of the RCC.…”

“…Cancer-associated retinopathy (CAR) is an important paraneoplastic syndrome to diagnose as it is not only rapidly progressive with poor visual recovery, but also visual symptoms precede other manifestations of an underlying malignancy [1]. The pathogenesis is thought to involve molecular mimicry generating antibodies against tumor cells, such as anti-recoverin, which then cross the blood-retina barrier to bind onto respective epitopes on photoreceptors, leading to caspase-dependent apoptosis [2].…”

Ocular Paraneoplastic Syndromes

“…This results in symptoms of cone dysfunction, namely, photosensitivity as well as photopsias (type of visual hallucinations seen as light flashes), prolonged glare, decreased best-corrected visual acuity (BCVA), color discrimination or disturbed color vision, and central scotomas. Rod dysfunction may include night blindness, prolonged adaptation to darkness, and peripheral or ring scotomas [ 4 , 5 , 6 , 7 , 8 , 9 , 10 , 11 , 12 , 13 , 14 , 15 , 16 , 17 , 18 ].…”

“…Central or ring scotomas should be detected in visual field examination. In electroretinography (ERG), rod- and cone-mediated responses are not recordable or significantly decreased, firstly affecting the a-wave and subsequently lining rapidly to a “flat ERG” [ 8 , 11 , 12 , 13 , 14 , 15 , 16 , 17 , 18 , 19 , 20 ].…”