Unilateral carcinoma‐associated retinopathy: diagnosis, serology and treatment

• Published in: Acta ophthalmologica (Oxford, England) Vol. 94; no. S256
• Main Authors: Roels, D., Ueno, S., Kondo, M., Leroy, B.P.
• Format: Journal Article
• Language: English
• Published: Malden Wiley Subscription Services, Inc 01.10.2016
• Subjects: Adenocarcinoma; Autoantibodies; Bipolar cells; Case reports; Chemotherapy; Clearing; Color vision; Corticoids; Corticosteroids; Diagnosis; Electronegativity; Electroretinograms; Hysterectomy; Immunoglobulins; Metastases; Monoclonal antibodies; Ophthalmology; Retinopathy; Rituximab; Serology; Steroid hormones; Steroids; Surgery; Transient receptor potential proteins
• ISSN: 1755-375X; 1755-3768
• DOI: 10.1111/j.1755-3768.2016.0428

Purpose To report a case of unilateral CAR with clinical and ERG normalization after radical hysterectomy combined with steroids and Rituximab. Methods Work‐up included extensive clinical and electrophysiological testing. Also, serological work‐up for antiretinal antibodies and oncological screening was organized. Results A 45‐year‐old female presented with progressive unilateral photopsias, photophobia and relative central scotoma in the RE since 6 weeks prior. BCVA was 1.0 in BE. Biomicroscopy, IOP and fundus exam were unremarkable. Also, colour vision, AFI, OCT and EOG were normal. VFs showed decreased central sensitivity in the RE. ERG showed an electronegative combined and ON‐bipolar response. A diagnosis of CAR was suspected. Radical hysterectomy was performed after a diagnosis of an undifferentiated cystic adenocarcinoma of the right ovary, followed by adjuvant chemotherapy. A whole body PET scan revealed no metastasis. Treatment with rituximab monoclonal antibodies in combination with corticosteroids was initiated. The patient tested positive for serum auto‐antibodies against TRPM1, a transient receptor potential cation channel expressed in ON‐bipolar cells. After treatment there was progressive improvement in symptoms and the ERG normalised. Serology confirmed complete clearance of auto‐antibodies. Conclusions Although extremely rare, unilateral CAR does occur and in cases with high clinical suspicion an oncological work‐up is mandatory. Aggressive treatment combining surgery, steroids and Rituximab can lead to clinical and ERG normalization with clearing of antiretinal antibodies.