Abstract

Introduction: Paraneoplastic syndromes are usually associated to small-cell lung carcinomas (SCLC) and are frequently due to the production of auto-antibodies. We report the case of a rare paraneoplastic syndrome, the Cancer-Associated Retinopathy (CAR) syndrome. Case report: A 66 years-old woman consulted her ophtalmologist for rapid-onset bilateral visual loss, nyctalopia and metamorphopsia. The visual acuity was 6/10 and 2/10 in the right and left eye, respectively. Diagnosis was an auto-immune retinopathy, with the presence of anti-recoverin antibody. The chest CT-scan revealed a pulmonary nodule in the right upper lobe. After complete evaluation, a SCLC with CAR syndrome was diagnosed. The patient underwent concomitant radiochemotherapy by Cisplatin/Etoposid, with a complete response at the end of the treatment course. Initially, the retinopathy was treated by a 3-day course of high-dose I.V. corticosteroid (500mg methylprednisolone per day) followed by oral prednisone at 1mg.kg daily and slow decrease. The CAR syndrome relapsed at 32 month of treatment without cancer relapse, and required to re-increase corticosteroid treatment. Review of literature: In a series of 209 patients with Cancer-Associated Retinopathy and Melanoma-Associated Retinopathy (MAR), Adamus et al. found that cancer associated with CAR syndrome were mainly breast (31%), lung (16%) and hematological malignancies (15%). In 31 lung cancer, 15 patients had auto-antibodies, most frequently anti-recoverin. Conclusion: CAR syndrome is a rare entity, which led, in this case, to the diagnosis of a limited SCLC. The CAR syndrome can relapse independently of cancer evolution.

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