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.
Highlights
The early diagnosis of cancer is of crucial importance and a key prognostic factor
Cancer-Associated Retinopathy (CAR) is a paraneoplastic, autoimmune retinopathy characterized by diffuse retinal degeneration
Cancer-associated retinopathy (CAR) is associated with a variety of cancers, among which small cell lung carcinoma are the most frequent [1]
Summary
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. Cancer-Associated Retinopathy (CAR) is a paraneoplastic, autoimmune retinopathy characterized by diffuse retinal degeneration. CAR is associated with a variety of cancers, among which small cell lung carcinoma are the most frequent [1]. The malignancy associated with a CAR can be diagnosed before or after the onset of the ocular manifestations. Because CAR is rare, the descriptions of the disease are based on small series and case reports. As cancer research progresses with improved treatments and increased survival rates, the long term visual outcome of CAR can be assessed
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