Jerry A. Shields
BASICS
DESCRIPTION
• Most common intraocular tumor
• Can occur within the eye in the uveal tissue including the iris, ciliary body, or choroid
• Appears as a pigmented (80%) or nonpigmented (20%) mass
• Generally measures about 2 mm in diameter and 1.5 mm in thickness
• Can produce overlying changes in the retinal pigment epithelium (RPE) such as RPE hyperplasia, atrophy, and drusen
ALERT
Choroidal nevus is a benign mass in the ocular fundus, but the patient should be evaluated to rule out simulating choroidal melanoma.
Geriatric Considerations
Choroidal nevus can occur in all ages.
Pediatric Considerations
Choroidal nevus can be present in children but often without drusen.
Pregnancy Considerations
There are no pregnancy considerations.
EPIDEMIOLOGY
Incidence
• Far more common in Caucasians than non-Caucasians
• Believed to be equivalent between male and female, although some studies show more common in females.
• Most authorities believe that the prevalence is approximately 7% of Caucasian adults.
• In population-based studies, choroidal nevus prevalence has varied including
– 1.9% of persons over age 13 years
– 3.1% of persons over age 30 years
– 6.5% of persons over age 49 years
Prevalence
• It is believed that choroidal nevus is present at birth but is recognized later in life after it acquires pigmentation or the patient cooperates for a dilated examination
• Age of onset:
– Young (≤20 years) in 2%
– Middle adult (20–50 years) in 23%
– Older adult (>50 years in) 75%
RISK FACTORS
Caucasian blue-eyes
Genetics
No hereditary tendency
GENERAL PREVENTION
There is no method for prevention of choroidal nevus.
PATHOPHYSIOLOGY
No known cause
ETIOLOGY
No known cause
COMMONLY ASSOCIATED CONDITIONS
• Ocular and oculodermal melanocytosis classically show diffuse pigmentation of the uvea (and choroid) consistent with a large flat nevus.
• Neurofibromatosis type 1 can manifest diffuse multifocal nevi.
DIAGNOSIS
HISTORY
• Decrease in visual acuity in 10%
• Flashes or floaters in 1%
• Visual field defect in 5%
• No symptoms in 84%
PHYSICAL EXAM
• Color is brown (80%) or yellow (20%)
• Location is subfoveolar (10%) or extrafoveolar (90%)
• Sector quadrant is macula (25%), inferior (20%), temporal (20%), superior (15%), nasal (20%)
• Anteroposterior quadrant macula (25%), macula to equator (70%), equator to ora serrata (5%)
DIAGNOSTIC TESTS & INTERPRETATION
Lab
Initial lab tests
No blood laboratory tests needed
Follow-Up & Special Considerations
No systemic follow-up as this is localized to the eye.
Imaging
Initial approach
• Ophthalmic Imaging includes: Fundus photography, ultrasonography, optical coherence tomography, fluorescein angiography, indocyanine green angiography, autofluorescence, and occasionally visual fields
– Ophthalmoscopy – dilated eye examination with indirect ophthalmoscopy and tumor measurement
– Fundus photography – to confirm the location and size of the tumor and its effects on the surrounding fundus
– Ultrasonography – imaging of the globe using sound waves to detect intraocular mass or retinal detachment to resolution of 0.5 mm
– Optical coherence tomography – imaging of the retina cross section to 8 micrometer resolution using light reflection to visualize the overlying retina to evaluate for subretinal fluid, photoreceptor loss, intraretinal edema, pigment epithelial detachment, choroidal neovascularization
– Fluorescein angiography – imaging of the retina and choroidal vasculature to evaluate for RPE atrophy or hyperplasia, choroidal neovascular membrane, and intrinsic choroidal tumor blood flow
– Indocyanine green angiography – imaging of the choroidal vasculature to evaluate for intrinsic tumor blood supply as well as choroidal neovascular membrane
– Autofluorescence – photographic imaging technique to evaluate for intrinsic RPE, choroidal, and scleral autofluorescence using special filters to detect emitted wavelengths of light after stimulation with light
– Visual fields – o evaluate for focal or diffuse loss of peripheral or central vision
Follow-up & special considerations
At each visit, some or all of the above tests are performed.
Diagnostic Procedures/Other
If suspicious, a fine needle aspiration biopsy for cytology or cytogenetics can be performed.
Pathological Findings
• Variably pigmented placoid choroidal lesion
• Involves outer choroid and usually spares the choriocapillaris
• Typically <2 mm thickness
• 4 histopathologic types include
– Plump polyhedral cells with round to ovoid small uniform nuclei
– Slender spindle cells with thin basophilic nuclei
– Plump fusiform and dendritic cells
– Balloon cells, found in 4% of nevi and have foamy cytoplasm
DIFFERENTIAL DIAGNOSIS
• Choroidal melanoma
• Congenital hypertrophy of the RPE
• RPE hyperplasia
• Choroidal metastasis from skin melanoma
• Subretinal hemorrhage
• Choroidal hemorrhage
• Peripheral exudative hemorrhagic chorioretinopathy
• Age related macular degeneration
TREATMENT
MEDICATION
There are no medications
ADDITIONAL TREATMENT
General Measures
• Observation on 3–6 month basis depending on risk factors
• Fundus photography to confirm appearance
• Ultrasonography to measure thickness
• Optical coherence tomography to confirm the features of the overlying retina
• Autofluorescence to evaluate for status of the RPE
Issues for Referral
• If risk factors present, then referral advised. Nevi with <3 risk factors could be observed. Nevi with >3 factors should be considered for treatment as they might represent melanoma. Risk factors include:
– To – thickness >2 mm
– Find – subretinal fluid
– Small – symptoms
– Ocular – Orange pigment
– Melanoma – Margin within 3 mm of disc
– Using Helpful – Ultrasound Hollow
– Hints – Halo absent
– Daily – Drusen absent
SURGERY/OTHER PROCEDURES
• If subretinal fluid into the fovea, consider laser photocoagulation or photodynamic therapy to the leaks detected on fluorescein angiography. Consideration for intravitreal bevacizumab to reduce subfoveal fluid can be made
• If documented growth of the mass, this could represent transformation into melanoma and plaque radiotherapy, thermotherapy, enucleation, or a combination of the above should be considered
IN-PATIENT CONSIDERATIONS
This is not managed as inpatient.
ONGOING CARE
FOLLOW-UP RECOMMENDATIONS
Patient Monitoring
Monitor in 3 months to document stability, then twice yearly for life.
PATIENT EDUCATION
PROGNOSIS
• Systemic – If stable nevus with no growth then systemic prognosis excellent
• Visual prognosis depends on tumor location:
– If the nevus is extrafoveal, then vision likely to be good
– If the nevus is subfoveal, then 26% risk for loss of at least 3 lines of visual acuity
COMPLICATIONS
• Loss of vision
• Transformation into melanoma
ADDITIONAL READING
• Ganley JP, Comstock GW. Benign nevi and malignant melanomas of the choroid. Am J Ophthalmol 1973;76:19–25.
• Sumich P, Mitchell P, Wang JJ. Choroidal nevi in a white population: The Blue Mountains Eye Study. Arch Ophthalmol 1998;116:645–50.
• Thiagalingam S, Wang JJ, Mitchell P. Absence of change in choroidal nevi across 5 years in an older population. Arch Ophthalmol 2004;122:89–93.
• Shields CL, Mashayekhi A, Materin MA, et al. Optical coherence tomography of choroidal nevus in 120 patients. Retina 2005;25:243–52.
• Shields CL, Shields JA, Kiratli H, et al. Risk factors for growth and metastasis of small choroidal melanocytic lesions. Ophthalmology 1995;102:1351–61.
• Shields CL, Cater JC, Shields JA, et al. Combination of clinical factors predictive of growth of small choroidal melanocytic tumors. Arch Ophthalmol 2000;118:360–4.
• Shields CL, Furuta M, Mashayekhi A, et al. Visual acuity in 3422 consecutive eyes with choroidal nevus. Arch Ophthalmol 2007;125:1501–7.
• Shields CL, Furuta M, Mashayekhi A, et al. Clinical spectrum of choroidal nevi based on age at presentation in 3422 consecutive eyes. Ophthalmology 2008;115:546–52.
• Collaborative Ocular Melanoma Study Group. Factors predictive of growth and treatment of small choroidal melanoma: COMS report no. 5. Arch Ophthalmol 1997;115:1537–44.
• Shields JA, Shields CL. Atlas of Intraocular Tumors. Philadelphia: Lippincott Williams and Wilkins, 2008:59–67.
• Shields JA, Shields CL. Intraocular Tumors: A Text and Atlas. Philadelphia: Saunders, 1992:85–100.
CODES
ICD9
224.6 Benign neoplasm of choroid
CLINICAL PEARLS
• Choroidal nevus is benign and should be monitored twice yearly.
• Choroidal nevus should be evaluated for clinical risk factors to ascertain whether or not it might show features of simulating malignant melanoma.
• Risk factors include:
– Thickness >2 mm
– Subretinal fluid
– Symptoms
– Orange pigment
– Margin within 3 mm of disc
– Ultrasound hollow
– Drusen absent
– Halo absent
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