Cancer Associated Retinopathy Related to Gastrointestinal Stromal Tumor of Stomach: Case Report

To report a patient who developed cancer associated retinopathy (CAR) in the setting of metastatic gastrointestinal stromal tumor (GIST) of stomach.

A 70-year-old man with the history of GIST presented to a tertiary eye center with blurry vision and gradually progressive nyctalopia in both eyes. Visual acuity (VA) was 20/40 in both eyes. Detailed investigations revealed progressive generalized constriction of visual fields with central scotomas and enlarged blind spots in both eyes, symmetrically reduced scotopic and photopic amplitudes on full field ERG, and positive antiretinal antibodies against carbonic anhydrase II and Rab6. Genetic testing was unrevealing and vitamin A level was within normal limits. As the preliminary diagnosis of CAR, intravitreal dexamethasone implants were administered in both eyes sequentially which resulted in significant improvement of visual fields. Systemic intravenous immunoglobulin (IVIg) therapy was started thereafter for the long-term management of CAR.

Although CAR is most seen in patients with carcinoma, the index case highlights that CAR may also be seen in a patient with GIST. Local therapy with intravitreal dexamethasone implant as the first line management may be supportive of a diagnosis of CAR, as well as useful given its rapid local effect while waiting for systemic assessment and therapy.