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Richard H. Schoen, OD
Clinical associate
Wilmer Eye Institute Suite 455 Lutherville, Maryland 21093 410-583-2800
February 2, 2015
Topic area:
Grand Rounds
Abstract
This is a case of 56 year old female presenting with sudden onset of blurry vision OD resulting from large uveoscleral melanoma. She is faced with challenging treatment options.
I. Case History

Patient demographics
o

Caucasian female, age 56. Married. Landscape architect.
Chief complaint
o
Right eye vision is worse recently and patient states she can’t use her progressive power lens
spectacles.

o
Rapid change in vision right eye, first noticed six weeks ago.
o
Red spot noted on exterior right eye, temporal bulbar conjunctiva
Ocular, medical history
o
Ocular history: compound hyperopic astigmatism and presbyopia. No apparent ocular pathology.
Depression. No hx of cancer. Last mammogram 1 year ago; normal.
Surgical history: Right oophorectomy
Family cancer history: Cancer Maternal Aunt breast cancer
Cancer Maternal Grandmother breast cancer
Smoking status: Former Smoker -- 0.50 packs/day for 5 years Quit date: 10/31/2014
Date of smoking status history with oncologist was 10/31/2014, same as quit date.


Medications
o
Alprazolam 1 mg three times a day for anxiety.
o
Risperidone 1 mg two times a day.
Other salient information
o
Penicillin allergy
II. Pertinent findings

Clinical 20/40 best corrected OD and 20/20 best corrected OS.


Pupil reactions intact. GAT 13 OD and 14 OS. Ocular motility intact OU.
Sentinel vessels inferotemporally OD (photo available)
Dilated fundus evaluation
(Photos available)
Epiretinal membrane OD. Inferior-temporal choroidal mass; pigmented; some orange pigment overlying; 10 mm
thickness x 12mm x13mm. Shallow overlying fluid. No exudative retinal detachment inferiorly.
Grade 1+ nuclear sclerosis OU.
OS: peripheral RPE changes; minimal
Physical evaluation with oncologist:


E. W. is a 56 y.o. female with choroidal melanoma.
1. The patient will see me every 3 months for careful monitoring. This will include labs, physical examinations
and regular radiologic imaging (CT chest with contrast and Liver MRI with and without Eovist every 3 months
for 2 years, then every 4 months for 2 years, then every 6 months for 2 years).

2. Chromosomal studies to evaluate risk of recurrence.

Laboratory studies, Oncologist’s orders, Open Future Orders:
Priority
Expected
Expires
Ordered
Complete Blood Count (CBC) + Auto Diff
Routine
5/30/2015
1/30/2016
1/30/2015
Comprehensive Metabolic Panel
Routine
5/30/2015
1/30/2016
1/30/2015
Lactate dehydrogenase (LDH)
Routine
5/30/2015
1/30/2016
1/30/2015
CT Chest/Abdomen/Pelvis W/ Contrast
Routine
5/30/2015
1/30/2016
1/30/2015
MRI Abdomen W/WO Contrast
Routine
5/30/2015
1/30/2016
1/30/2015
Oncology Appointment Request
Routine
5/30/2015
1/30/2016
1/30/2015
CT Chest/Abdomen/Pelvis W/ Contrast
Routine
1/15/2015
11/26/2015
11/26/2014
MRI Abdomen W/WO Contrast
Routine
1/15/2015
11/26/2015
11/26/2014
MRI Abdomen W/WO Contrast
Routine
10/31/2014
10/31/2015
10/31/2014

Radiology studies
CT SCAN OF THE CHEST, ABDOMEN AND PELVIS WITH CONTRAST
HISTORY: Choroidal melanoma, staging
TECHNIQUE: Computed tomography of the chest, abdomen and pelvis was
performed with 120 ml of Omnipaque 350 intravenous contrast. Oral
contrast was also administered. Staging with CT chest/abdomen/pelvis was negative for metastatic disease.
To date, the following imaging studies have been conducted. All are without evidence of metastasis:
CT scan of chest/abdomen/pelvis with contrast, monthly. MRI abdomen with and without contrast, monthly.
Chest X-ray perhaps every six months.

Others
Ultrasounography: large iridociliochoroidal melanoma in the right eye measuring 10.4mm height x 13.0mm x 12.4mm
(only the choroidal component), without extrascleral extension. (Image available).
III. Differential diagnosis
Choroidal hemorrhage, ocular melanoma, scleritis, uveitis
IV. Diagnosis and discussion

Elaborate on the condition
Large iridociliochoroidal melanoma 10 mm height x 13 x 12 mm base right eye
Laboratory tests as well as imaging indicate no evidence of metastasis. The eye is red, bulbar conjunctiva
injected with sentinel vessels. Best corrected acuity is reduced to 20/40 OD. The tumor can be visualized
through the dilated pupil.

Uveal melanoma has a predilection to metastasize to the liver and then elsewhere. It is associated with
significant 5 year mortality rate.i Treatment options including plaque brachytherapy (not recommended
given large size and extent of iris/ciliary body/choroid involvement) and enucleation (recommended)

Expound on unique features
The options were reviewed in general terms which include enucleation, plaque brachytherapy, proton radiation, and
stereotactic radiosurgery. While enucleation is recommended given the size and extent of her lesion, she has chosen
to proceed with plaque brachytherapy. Issues of clinical diagnosis and the risk of late vision loss associated with
plaque brachytherapy or proton irradiation were discussed.ii Given the thickness of the lesion, plaque brachytherapy is
typically contraindicated favouring proton irradiation or enucleation. After a long discussion, Ms. E. W. indicated a
wish to still proceed with an implant reserving enucleation for salvage. The long term concern would be whether
enucleation would reduce relative risk of metastasis. Additionally, large tumor size at presentation is associated with
increased potential for metastasis and perhaps for mortality.
Treatment and management
iridociliochoroidal melanoma, right eye 190.6.
CLINICAL STAGE OF TUMOR:
Large size by COMS size criteria.
TITLE OF OPERATION:
Insertion of iodine 125 radioactive plaque, 18 mm, disinsertion of the lateral rectus muscle with hang-back technique,
Fine-needle aspiration biopsy of tumor, right eye.
This takes place 24 days after discovery of the tumor.
General anesthesia
Fine needle aspiration biopsy of iridociliochoroidal melanoma for clinical genetic testing.
Device implant for brachytherapy (video is available)
Four days later:
TITLE OF OPERATION:
Removal of iodine 125 radioactive plaque (18 mm), reinsertion of the lateral rectus muscle using hang-back technique
in the right eye.
On day post op, distance visual acuity with pinhole 20/80. Patient reports bilateral diplopia.
Five days later, acuity OD distance with pinhole 20/100. Diplopia has subsided.
The wound is healing as expected.
Twenty days later, diplopia has abated.
Distance acuity OD with pinhole is 20/63-1
Today pt states vision has not improved. Pt states there is a color change OD, she states things appear "pinker." Pt
states OD is extremely sensitive to light, and the whole right side of her face has pain occasionally. Pt also states her
eye is watering often. Pt states OS is stable.
Most recent follow up with oncologist (54 days after brachytherapy).
FNAB [Fine Needle Aspiration Biopsy] (Castle Biosciencesiii): Class 2 tumor (high risk gene expression profile) iv; We
discussed regional adjuvant treatment trials in Philadelphia (Sunitinib vs Valproic acid) and New York (Crizotinib). She
prefers to defer enrollment and we will continue active surveillance with imaging. The patient and I discussed the
high-risk nature of the lesion and where one might expect a recurrence, should that occur. We also discussed the
experimental nature of the trials listed above.
Conclusion
Patients that report with sudden change in vision should have a meticulous dilated fundus examination. Ominous
pathology such as uveal melanoma is somewhat rare with between 4 and 7 cases per million incidence in the United
States. One mark of an effective clinician is being connected with a network of highly able tertiary care colleagues. It
is likely that the patient in the case report benefited, on the presenting visit, from same day ordering of ultrasound.
Once the tumor was imaged in this manner, the Retinal Oncology Specialist was prepared to direct the patient into
intervention without delay. The patient chose brachytherapy rather than enucleation. Sparing the eye for as long as
possible is important to this particular individual.
Arrangements were made so that the patient has tight and
meticulous surveillance by systemic oncologist with the hope that if metastasis occurs, it will be detected and treated
without delay.
The AJCC Ophthalmic Oncology Task Force. International Validation of the American Joint Committee on Cancer’s
7th Edition Classification of Uveal Melanoma. JAMA Ophthalmol. Published online January 02, 2015.
doi:10.1001/jamaophthalmol.2014.5395.
i
ii
http://myuvealmelanoma.com/wp-content/uploads/2012/12/Gill-2012.pdf
Harmeet S. Gill, MD, FRCSC*,‡, Devron H. Char, MD, FACS. Uveal melanoma prognostication: from lesion size and
cell type to molecular class. Can J Ophthalmol 2012;47:246–253
iii
http://med.miami.edu/news/bascom-palmer-eye-institute-researcher-identifies-new-genetic-mutation-in-o
Field MG1, Harbour JW. Recent developments in prognostic and predictive testing in uveal melanoma. Curr Opin
Ophthalmol. 2014 May;25(3):234-9
iv