1. 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

2. 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

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

4. 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

5. 20 March 2012: First Presentation
OCT: disruption of photoreceptor layer OU
FFA: mild early hyperfluorescence along the superior arcade.

6. 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.

7. 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.

8. 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

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

11. 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)

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

13. 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)

14. Presumptive Diagnosis
Cancer associated retinopathy

15. 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

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

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

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

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

20. 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

21. 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

22. 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