Cancer-associated retinopathy preceding the diagnosis of cancer

Abstract

Background: The 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.

Methods: This 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.

Results: Four 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.

Conclusion: In selected patients, findings suggestive of CAR can be useful for the early detection of a cancer.

Keywords: Cancer; Cancer-associated retinopathy; Paraneoplastic retinopathy; Uveitis.

Fig. 1

Spectral-domain optical coherence tomography (Spectralis, Heidelberg Engineering, Germany) showing the loss of the external limiting membrane, of the inner segment/outer segment junction and of the outer nuclear and plexiform layers with a sparing of the foveal region

Fig. 2

Fundus photography (Digital Non-Mydriatic Retinal Camera, Canon, USA) of patient four at the last follow-up. Diffuse retinal atrophy combined with arteriolar attenuation, optic disc pallor and pigment deposits are observed