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Choroidal mass noted in right eye of middle-aged woman
B-scan ultrasonography showed a minimally elevated lesion with high internal reflectivity.
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A 55-year-old woman was referred to the New England Eye Center for evaluation of a lesion in the right eye noted incidentally during an annual eye exam. She denied any change in vision, photopsias or eye pain. She had undergone uncomplicated cataract surgery in both eyes 1.5 years before presentation. She denied any other ocular history.
Her medical history was significant for stage 4 breast cancer with metastases to the brain, spine and contralateral breast. She was first diagnosed with breast cancer 17 years before presentation. She developed metastatic intracranial lesions and underwent whole brain radiation 2 years before presentation, followed by gamma knife radiation for new brain metastases over the past year. At the time of presentation, she was taking anastrozole (hormonal therapy) and capecitabine (chemotherapy). Family and social histories were noncontributory.
Figure 1. Mosaic color fundus photograph of the right eye showing a non-pigmented choroidal mass nasal to the optic disc.
Images: Muakkassa NW, Duker JS
Figure 2. B-scan of the right eye showing a mass with high internal reflectivity, measured to have a maximal thickness of 2 mm.
Figure 3. OCT images through the mass demonstrating choroidal elevation, a small amount of overlying subretinal fluid, retinal thickening and RPE detachment.
Examination
On examination, the patient’s best corrected visual acuity was 20/25 in both eyes. No afferent pupillary defect was detected. Confrontation visual fields were full in both eyes. IOP was 17 mm Hg in both eyes. Anterior segment examination was remarkable for pseudophakia bilaterally. Dilated funduscopic exam of the right eye revealed a slightly elevated non-pigmented choroidal mass with overlying mottling of the retinal pigment epithelium (RPE). The mass was nasal to the disc and measured approximately 10 mm by 11 mm in basal diameter (Figure 1). Funduscopic examination of the left eye was normal.
B-scan ultrasonography of the right eye showed a minimally elevated lesion with high internal reflectivity with maximal thickness of 2 mm (Figure 2). An optical coherence tomography image through the lesion showed minimal choroidal elevation with a small amount of overlying subretinal fluid, RPE detachment and retinal thickening (Figure 3).
What is your diagnosis?
Choroidal mass
The differential diagnosis for a unilateral, focal, non-pigmented choroidal mass includes amelanotic choroidal nevus, amelanotic choroidal melanoma, choroidal metastasis, choroidal granuloma and choroidal osteoma.
Amelanotic choroidal nevi are typically flat, although they may be slightly elevated. Amelanotic choroidal melanomas share features of typical choroidal melanomas but lack the classic dark brown pigmentation. They present as a choroidal mass that is usually greater than 2 mm in height. They may be associated with serous retinal detachments and are typically sonolucent on B-scan. Fundus fluorescein angiography may show a dual circulation, representing the tumor’s blood supply along with the normal retinal circulation.
Choroidal metastases present as unilateral, focal lesions in 80% of cases. The color of the mass depends on the type of primary malignancy and can range from yellow to orange to darkly pigmented. Choroidal metastases can look similar to amelanotic choroidal melanomas on funduscopic exam. B-scan is useful to distinguish the two; metastases typically have high internal reflectivity whereas melanomas are usually sonolucent.
Choroidal osteomas are benign, rare bony tumors of the choroid. They are typically unilateral, peripapillary in location and yellow/orange in color with well-defined borders. They are usually relatively flat but may be elevated. B-scan shows high reflectivity with shadowing, consistent with bone.
Diagnosis and management
The B-scan findings were helpful in making the diagnosis in this case. The lesion was sonoreflective, making a melanoma less likely. The B-scan findings were not consistent with bone, ruling out an osteoma. The size, height and presence of subretinal fluid make a benign diagnosis such as a nevus unlikely. We suspected a choroidal metastasis as the most likely diagnosis.
Given the history, clinical findings and imaging, the patient was diagnosed with a choroidal metastasis secondary to breast cancer. It was presumed to be inactive given its relatively minimal thickness, minimal amount of overlying subretinal fluid and significant RPE changes.
Discussion
Choroidal metastases are the most common malignant intraocular tumor in adults. The lesions are solitary and unilateral in 80% of cases but may also present as multifocal or bilateral lesions. There is a known diagnosis of cancer 75% of the time. Most metastases are yellow in color. Metastases that are orange in color should raise suspicion for renal cell carcinoma, follicular carcinoma of the thyroid and carcinoid tumors. Of patients with known extraocular malignancy, the most common primary malignancy is breast in women and lung in men.
Diagnosis is made primarily by features on examination and B-scan. As discussed above, B-scan images of choroidal metastases typically show high internal reflectivity, helping to distinguish it from amelanotic choroidal melanomas.
If there is a known primary malignancy, the patient should have a full systemic evaluation and staging with his or her oncologist after detection of the choroidal metastasis. If there is no known primary, a thorough systemic evaluation is indicated. This includes a complete physical exam, mammography, chest radiograph, and full-body CT or PET scan. If the work-up is unrevealing, a biopsy is indicated to identify the primary site of malignancy.
Treatment options depend on the location of the tumor and its effect on visual acuity. Macular tumors or tumors causing visually significant serous retinal detachments can be treated with radiation therapy. Options include plaque therapy or external beam radiation. If applicable, systemic chemotherapy and/or hormonal therapy should be employed. These tumors are typically responsive to therapy, and regressed tumors often show overlying RPE changes. Painful eyes with poor visual potential may be enucleated.
Choroidal metastases are poor prognostic indicators of survival. The estimated survival rates in patients with uveal metastases from breast cancer are 65% at 1 year and 24% at 5 years.
References:
Augsburger JJ, Guthoff R. Metastatic cancer to the eye. In: Yanoff M, Duker JS, eds. Ophthalmology. 3rd ed. Elsevier; 2009:906-910.Demirci H, et al. Am J Ophthalmol. 2003;doi:10.1016/S0002-9394(03)00192-2. Shields CL, et al. Arch Ophthalmol. 1997;doi:10.1001/archopht.1997.01100150205010. Shields CL, et al. Ophthalmology. 1997;doi: 10.1016/S0161-6420(97)30148-1.