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

2
Medical History

Diabetes Mellitus type 2

2010
 endometrial carcinoma s/p hysterectomy +
chemotherapy + radiation therapy;
 cured 2012
3
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
4
20 March 2012: First Presentation

OCT: disruption of photoreceptor layer OU

FFA: mild early hyperfluorescence along the
superior arcade.
5
OCT of both eyes show disruption of photoreceptor layer.
Fundus photos are within normal.
6
Fluorescein angiography of both eyes shows mild early
hyperfluorescence along the superior arcade.
7
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
8
9
10
ERG Interpretation

ERG:
 OD: prolonged implicit time of scotopic rod
response
 OS: attenuation of photopic cone response
(above figures/slides 9 and 10)
11
Work-up

ESR: 25 mm

CRP:6.6 mg/dL

PPD skin test: negative
12
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)
13
Presumptive Diagnosis
Cancer associated retinopathy
14
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
15
Management

Plan by gynecologist:
 another cycle of radiotherapy

Plan by ophthalmologist:
 Prednisone 40mg orally daily,
 Cellcept: 500 mg PO bid
16
Follow-up
January 2013
 Improved VA: OD 20/200, OS 20/120

Improved color vision: 7/24 OU

Fundus: Normal OU

FA: Normal OU
17
Normal fundus photos and fluorescein angiography of both eyes.
18
Follow-up
January 2013
 Anti-retinal autoantibodies: positive against 25-kDa,
30-kDa, 34-kDa, 36-kDa, and
46kDa(enolase)

Neuron Specific Enolase, S: 6.6 ng/ml
(normal<15ng/ml)
19
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
20
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
21
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
22