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Case Report
The Case of the Dangerous Nevus
Elizabeth Walsh, O.D., James H. Quillen VAMC Optometry Resident
Abstract
Longstanding patient followed for normal tension glaucoma develops growth in a unilateral
juxtapapillary choroidal nevus with a corresponding visual field defect. He is referred for
choroidal melanoma and undergoes radioactive iodine brachytherapy plaque treatment.
I. Case History
72 year old white male complains of a gradual decreasing vision in the left eye. His systemic
health diagnoses includes type 2 insulin-dependent diabetes mellitus, hypertension,
hyperlipidemia, benign prostate hyperplasia, joint pain, osteomyelitis, extrasystole, dyspnea,
erectile dysfunction, keratoderma, and arthritis. His oral medications are Furosemide, Insulin,
Tamsulosin, Atneolol, Atorvastatin, Buspirone, Finasterid, Trazodone, Lorazepam, Methadone,
Gabapentin, Hydrochlorothiazide, and Fosinopril. He has an ocular history of pseudophakia with
YAG capsulotomy OU, normal tension glaucoma s/p ALT OU, treated with Latanoprost 0.005%
qhs OU, dry eye syndrome relieved with Refresh Tears 0.5% qid, and peripapillary choroidal
nevus OS. His first eye exam at the clinic was 2004 and has since been followed with yearly
OCTs of the optic nerve, Humphrey visual fields, and fundus photos.
II. Pertinent Findings
Between 2004 and 2013, exam notes document increased cupping and stable intraocular
pressures. Vision fluctuates, but most recently was 20/20 with correction. OCTs of the optic
nerve document more thinning of the right eye in superior, inferior, nasal quadrants as compared
to just borderline thinning temporally in the left eye. Photos show increased growth from 2004 to
2011 of the superior peripapillary nevus in the left eye. Humphrey visual fields show varying
defects in both eyes in a possible glaucomatous pattern. In regards to the choroidal nevus, an
abnormal OCT was noted on August 2013 with increased superior thickness in the left eye and
possible retinal fluid within the lesion. An HVF that month also showed a possible increase in
the patient’s blind spot of the left eye. A referral was made to an ophthalmology private
practice. The assessment was a medium 2 DD H/3DD V elevated superior peripapillary nevus
with a plan to refer to retina. He was seen by the same retina specialist in September 2013 and
March 2014 with a diagnosis of a suspicious nevus. One year after the first visit with retina, he
was diagnosed with stage 1 choroidal melanoma, secondary to documented growth. OCT shows
obliteration of the superior rim of the optic nerve, as well as a repeatable visual field defect
inferior temporal of the left eye. Comparison of fundus photos from 2011 to 2014 show definite
growth with elevation and infiltration into the optic nerve. A referral was made to Vanderbilt
Medical Center in Nashville, TN and an order was placed for a CT of orbits, MRI of the liver,
and GGT lab work to rule out metastatic cancer.
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CT of the orbits in October 2014 show “subtle modestly enhancing 4 mm lentiform lesion along
the inner wall of the posterior left globe, just superior to the optic disc, compatible with reported
choroidal melanoma.” Lab work, including creatinine and urea nitrogen levels, was normal at
both October and November 2014 visits. Data from the first Vanderbilt visit in November 2014
include visual acuities of 20/25 OD, 20/20 OS, intraocular pressures of 14 OU, and 0.75 cup-todisc ratio. A “juxtapapillary elevated, lightly pigmented lesion s/n, abutting ONH head, 50%
surrounded by tumor with blurring of superior half of nerve” was noted. Ancillary testing
included A- and B-scan, fundus auto-fluorescence and OCT of the lesion. The lesion was 2.5
mm height, 9.06 mm in diameter with low internal reflectivity. Hyperauto-fluorescence was
observed from overlying orange pigment and OCT showed subretinal fluid.
III. Differential Diagnosis
Choroidal nevi are described as flat or minimally elevated lesions, which can be pigmented or
amelanotic. They are typically less than 2 mm in thickness and might have gradual elevation.
Drusen may become more prominent with age. RPE atrophy, hyperplasia and detachment might
occur, but lipofuscin and subretinal fluid are rarely detected. Growth less than 1 mm can occur,
but if greater than 1 mm of growth in one year is documented, then the lesion should be followed
closely for melanoma. The rate of transformation from nevi to melanoma is 2% over 1 year, but
increases to 13% over a 10-year period. Choroidal melanoma is the most common primary
intraocular tumor. It typically is unilateral and findings including drusen, orange pigment or
lipofuscin, and exudative RD. Once the lesion reaches greater than 4mm thickness, it is thought
to be actively growing. 25% can be amelanotic and it has been reported that 1-14% of all lesions
will have optic nerve invasion, with 54-62% of those being peripapillary.
Differential diagnoses can be categorized into non-pigmented and pigmented categories,
depending on the characteristic of the choroidal lesion. For non-pigmented lesions, there are 6
possible differentials. Choroidal hemanigoma will have a red-orange appearance and lymphoma
findings include yellow-orange infiltration. Metastatic carcinoma will be cream or light brown
with extensive fluid. It can be multifocal or bilateral and probably originate from breast or lung
cancer. Choroidal osteoma has a yellow-orange appearance and is at the disc. It might have
pseudopod-like projections and typically affects young women. Posterior scleritis will have
choroidal folds, pain, proptosis, and other inflammatory signs, including the T Sign on
ultrasound. Lastly, sclerochoroidal calcification is typically yellow-white subRPE or choroidal
plaques, and is most often seen in the elderly. For pigmented lesions, a choroidal nevus is the
main differential. Congenital hypertrophy of the RPE (CHRPE) presents as flat, black lesions
with crisp margins and overlying lacunae. Also, reactive hyperplasia of the RPE can occur after
trauma or inflammation. Findings include a flat, black lesion with irregular margins and white
gliosis. Melanocytoma of the optic nerve head is another black lesion with fibrillated margins.
Growth may occur up to 15%. Lastly, choroidal detachment may appear as a dark, peripheral,
multi-lobular fundus mass.
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IV. Diagnosis and Discussion
Choroidal melanoma rarely occurs in African Americans. It is more commonly seen in light
pigmented individuals. A patient may present asymptomatically, but symptoms include
decreased vision, visual field defects, floaters, and flashes of light. Upon exam, a graygreen/brown or yellow mass with subretinal fluid and orange pigment will be present. It should
be thicker than or equal to 2 mm. The lesion may be a dome, mushroom, or plateau shape with
congested vessels in the apex. A break in Bruch’s membrane may occur and if followed over
time, growth will be noted. Risk factors for choroidal melanoma include tumor thickness >2mm,
subretinal fluid, symptoms, orange pigment, tumor margin within 3 mm of the optic disc,
ultrasonography hollowness, and an absence of a halo. This is easily remembered by the
mnemonic “To find small ocular melanoma using helpful hints.”
When a patient presents with a suspicious lesion, the following diagnostic tools must be used as
a baseline: binocular ophthalmoscopy with a 20D lens, fundus photography, OCT, autofluorescein angiography, and ultrasound for internal acoustic features. OCT is great to determine
the presence of retinal fluid and to visualize retinal features around the lesion. FA’s role is to
describe any lipofuscin or RPE disturbance.
V. Treatment and Management
In January 2015, the patient underwent radioactive iodine brachytherapy plaque treatment. A 14
mm plaque was inserted into the eye with I-125 seeds. Seven days later, the plaque was removed
and transpupillary thermotherapy (TTT) was used to destruct the lesion. At his 2 month followup, the tumor was noted as regressing nicely. At 5 months, he complained of double vision when
looking to the right, but his vision was 20/25 OD, OS. At 6 months, vision was still good, but
the visual field defect remained. Repeat CT scans in August showed a possible new lesion on
the lung and high levels of BUN, which might correlate with poor kidney function. Repeat CT
scans, HVF, and OCT will be performed later this year.
Other treatment options for choroidal melanomas include observation, internal beam radiation
and enucleation. The Collaborative Ocular Melanoma Study (COMS) is an international, multicentered study that determined the best treatment, based on size of the tumor. For small tumors
(>5-16 mm basal dimension with 1.5-3.0 mm apical height), observation proved to be the best
treatment with 31% growth in 5-years and no melanoma-specific deaths reported. For medium
tumors (>5-16 mm basal dimension with 2.5-10.0 mm apical height), both radioactive
brachytherapy and enucleation had similar survival rates, which ranged from 57%-59% over 12
years. Lastly, for large tumors (>16 mm basal dimension with >10.0 mm apical height), there
was a similar 10-year mortality rate between enucleation and enucleation with PERT. Their
conclusions include predictive growth factors, such as increased thickness/diameter and
increased age increases death rate, orange pigment, and drusen/RPE changes. Also, observation
should occur if the lesion is a small tumor and that pre-enucleation radiotherapy is not effective.
Lastly, for medium-sized tumors, radiotherapy should be utilized rather than enucleation.
Although the COMS study set precedence for melanoma treatment, it excluded cases related to
juxtapapillary melanomas. Choroidal malignant lesions adjoining the optic disc did not receive
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radiotherapy, but rather opted for enucleation. Another article discussed plaque radiotherapy for
choroidal melanoma that were juxtapapillary within less than 1 mm to the optic disc. A
retrospective case series was completed at the Wills Eye Hospital of 650 consecutive eyes,
comparing complications and outcomes of plaque therapy with and without transpupillary
thermal therapy. At the end of 10 years, the following findings were noted in patients after
plaque therapy: non-proliferative retinopathy (75%), proliferative retinopathy (32%),
maculopathy (65%), papillopathy (77%), cataract (80%), neovascular glaucoma (22%), vitreous
hemorrhage (42%), and enucleation (26%). At 5 years, enucleation was 16%, which is similar to
the COMS study of 13%. When TTT was utilized, macular and optic disc disease was noted
more (65% v 46%), but enucleation was lower (10% v 21%). However, when no TTT was used,
incidence of neovascular glaucoma was lower (9% v 19%). It was concluded from the study
that plaque radiotherapy should be utilized in the treatment of choroidal melanomas, due to the
high rate of eye retention, and the use of TTT cause similar outcomes.
Lastly, patients should continue to be followed regularly to document any signs of metastases.
The risks of this happening include older age, larger tumor size, extrascleral extension and ciliary
body involvement. The sites most often affected are the liver, lungs, and bone. Frequent CT
scans and liver enzyme lab work should be performed. Once metastases occur, there is typically
a 5-7 month survival rate.
VI. Conclusion
In conclusion, when you suspect a malignant choroidal lesion, observation is the first line of
treatment. A patient should be followed every 3-4 months until stability is confirmed. Once
stable, follow-ups should continue on a 6 month to 1 year basis to document lack of change. The
patient should be seen more often if they are deemed to be at higher risk. If 3 or more risk
factors are noted, it is important to refer out. The chance of a choroidal nevus transforming to a
more dangerous lesion is small, but it is important to not ignore the findings, because proper
treatment could be the difference between life and death.
VII. Resources
1. Messner, L. Ocular Melanoma. Powerpoint Lecture Summer 2013. Illinois College of
Optometry.
2. The Wills Eye Manual: Office and Emergency Room Diagnosis and Treatment of Eye
Disease 6th Edition. Editors: A. Gerstenblith and M. Rabinowitz. 2012.
3. Sagoo, M.S., et. al. Plaque Radiotherapy for Juxtapapillary Choroidal Melanoma:
Treatment Complications and Visual Outcomes in 650 Consecutive Cases JAMA
Ophthalmol. 2014;132(6):697-702
4. Materin, M.A., et. al. Fundus Autofluorescence and Optical Coherence Tomography
findings in Choroidal Melanocytic Lesion. Middle East Afr J Ophthalmol. 2010 July-Sep;
17(3): 201-206
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5. Diener-West, M., et. Al. Collaborative Ocular Melanoma Study Group. The COMS
Randomized Trial of Iodine 125 Brachytherapy for Choroidal Melanoma, III: Initial
Mortality Findings. COMS Report No. 18. Arch Ophthalmol. 2001 Jul.
6. Shields, C.L., et. al. Choroidal Nevus Transformation into Melanoma: Analysis of 2514
Consecutive Cases. Arch Ophthal. 2009 Aug;127(8):981-7
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