Cancer Associated Retinopathy Mahmoud O. Jaroudi, MD1, Khalid F. Tabbara, MD1,2,3 1The Eye Center and The Eye Foundation for Research in Ophthalmology, Riyadh, Saudi Arabia 2Department of Ophthalmology, College of Medicine, King Saud University, Riyadh, Saudi Arabia 3The Wilmer Ophthalmological Institute of the Johns Hopkins University School of Medicine, Baltimore, Maryland, USA

Ocular History 58 year old woman 20 March 2012: sudden decrease in vision OU of 1 month diagnosed elsewhere as case of uveitis treated with systemic steroids

Medical History Diabetes Mellitus type 2 2010 endometrial carcinoma s/p hysterectomy + chemotherapy + radiation therapy; cured 2012

20 March 2012: First Presentation VA: 20/400 OU IOP: 18 mmHg OD, 19 mmHg OS Clear corneas A/C: Deep and quit OU Fundus: clear vitreous OU, sheathing of one blood vessel inferonasal to optic nerve OD

20 March 2012: First Presentation OCT: disruption of photoreceptor layer OU FFA: mild early hyperfluorescence along the superior arcade.

MODIFIED JUNE 3, 2014 SLIDES WITH CAPTIONS CASE REPORT CAR April 26, 2017 OCT of both eyes show disruption of photoreceptor layer. Fundus photos are within normal.

MODIFIED JUNE 3, 2014 SLIDES WITH CAPTIONS CASE REPORT CAR April 26, 2017 Fluorescein angiography of both eyes shows mild early hyperfluorescence along the superior arcade.

20 March 2012: First Presentation Color vision: 0/24 plates OU ERG: OD: prolonged implicit time of scotopic rod response; OS: attenuation of photopic cone response Amsler test: Normal OU EOG: Normal OU VEP: Normal OU

MODIFIED JUNE 3, 2014 SLIDES WITH CAPTIONS CASE REPORT CAR April 26, 2017

MODIFIED JUNE 3, 2014 SLIDES WITH CAPTIONS CASE REPORT CAR April 26, 2017 ERG Interpretation ERG: OD: prolonged implicit time of scotopic rod response OS: attenuation of photopic cone response (above figures/slides 9 and 10)

Work-up ESR: 25 mm CRP:6.6 mg/dL PPD skin test: negative

Work-up April 2012 Anti-retinal autoantibodies: positive against 25-kDa, 40-kDa, 48-kDa, and 52-kDa proteins Neuron-specific Enolase, S: 7.8ng/ml (normal </= 15ng/ml)

Presumptive Diagnosis Cancer associated retinopathy

Work-up PET-scan to rule out recurrence of endometrial carcinoma. PET scan: enlarged abdominal lymph nodes (LN) with uptake Core needle biopsy of abdominal LN: involved by carcinoma

Management Plan by gynecologist: another cycle of radiotherapy Plan by ophthalmologist: Prednisone 40mg orally daily, Cellcept: 500 mg PO bid

Follow-up January 2013 Improved VA: OD 20/200, OS 20/120 Improved color vision: 7/24 OU Fundus: Normal OU FA: Normal OU

MODIFIED JUNE 3, 2014 SLIDES WITH CAPTIONS CASE REPORT CAR April 26, 2017 Normal fundus photos and fluorescein angiography of both eyes.

Follow-up January 2013 Anti-retinal autoantibodies: positive against 25-kDa, 30-kDa, 34-kDa, 36-kDa, and 46-kDa(enolase) Neuron Specific Enolase, S: 6.6 ng/ml (normal<15ng/ml)

Follow-up February 2014 Improved visison VA: 20/120 OU Anti-retinal autoantibodies: positive against 25-kDa, 30-kDa, 66-kDa, and 68-kDa proteins

Conclusions Cancer associated retinopathy (CAR) is a rare disease that can be easily overlooked when the clinical exam is normal It can be the initial manifestation of a new or recurrent systemic malignancy Diagnosis is confirmed by the presence of anti-retinal autoantibodeis and changes on ERG

Conclusions A shift in type of autoantibodies was observed during the course of the disease Immunomodulating therapy for CAR may pose a dilemma as it can also suppress anti-tumor cells when the primary malignancy is still active Treatment may not be effective in restoring normal vision, probably due to irreversible photoreceptor damage