Patient presents with blurry vision in right eye

An elevated amelanotic choroidal lesion was seen on examination.

Priti Batta

Namrata Nandakumar

A 64-year-old woman presented to the New England Eye Center with 2 weeks of blurred vision in her right eye. She denied any recent trauma, floaters, flashes or pain. She had no visual complaints in her left eye.

On exam, her best corrected visual acuity was 20/80^-2 in the right eye and 20/25 in the left eye. Pupils were equal and reactive to light with no afferent pupillary defect. Extraocular movements and confrontation visual fields were full bilaterally. IOP was 14 mm Hg in the right eye and 15 mm Hg in the left eye.

The patient’s anterior segment exam showed trace nuclear sclerotic cataracts bilaterally. Her dilated funduscopic examination revealed clear media bilaterally. Both optic nerves had normal cup-to-disc ratios with sharp disc margins and normal color. Retinal vessels were normal in color and caliber bilaterally. In the right eye, the foveal reflex was blunted with retinal pigment epithelial changes. A well-circumscribed elevated amelanotic choroidal lesion, 8 mm × 9 mm wide, was noted superonasally adjacent to the optic disc. Few dot-blot hemorrhages were seen peripherally (Figure 1). The dilated fundus exam of the left eye was normal.

Figure 1. Mosaic photograph of right eye. Blunted foveal reflex with retinal pigment epithelial changes. A well-circumscribed elevated amelanotic choroidal lesion, 8 mm x 9 mm wide, is located superonasally adjacent to the optic disc. Few dot-blot hemorrhages are seen peripherally. Images: Vuong LN, Duker JS

What is your diagnosis?

Choroidal lesion

Based on the clinical exam alone, the differential diagnosis for a well-circumscribed elevated amelanotic choroidal lesion is relatively narrow: amelanotic melanoma, choroidal granuloma and choroidal metastasis.

A B-scan of the right orbit showed a solid 2-mm elevated mass (Figure 2). Optical coherence tomography of the fovea revealed subretinal fluid with a central foveal thickness of 724 µm (Figure 3). Fluorescein angiography showed early hypofluorescence (Figure 4a) and late hyperfluorescence of the lesion with leakage into the fovea (Figures 4b and 4c).

Figure 2. B-scan of right eye. Solid mass elevated by 2 mm is seen in the posterior pole.

Figure 3. OCT of the right eye. Subretinal fluid is seen in the fovea with a central foveal thickness measurement of 724 µm.

Figure 4. Fluorescein angiography of right eye. A. Early fluorescein angiography images show hypofluorescence of the mass with no vasculature. B. Late fluorescein angiography images show hyperfluorescence of the lesion and C. leakage in the fovea.

Most importantly, the patient’s medical history revealed a diagnosis of cholangiocarcinoma. The patient was diagnosed in 2008, had two failed chemotherapy treatments, and had metastases to the liver and lungs. The patient also had a history of basal cell carcinoma and endometrial cancer, for which she underwent a hysterectomy. At the time of examination at the New England Eye Center, she was scheduled to undergo chemotherapy for metastatic cholangiocarcinoma.

Diagnosis

Choroidal metastasis can be the presenting sign of a metastasis from a primary cancer in up to one-third of patients with cancer. The most common primary malignancy is breast cancer in women and lung cancer in men. Survival is limited in patients after ocular metastasis is discovered, with a range of 5 months to 20 months, slightly longer in patients with breast cancer.

The diagnosis of choroidal metastasis is based mainly on medical history and clinical exam findings. The majority of metastases are unilateral and unifocal, but it is not uncommon for them to be multifocal and bilateral. These masses are characteristically round or oval placoid in shape. Depending on the primary malignancy, the metastases have different colors: Breast and lung metastases are pale yellow; cutaneous melanoma metastases are dark gray or brown; renal cell and thyroid carcinoma metastases are orange-red; and metastatic carcinoid is pink or yellow-orange.

Typically, B-scans show an acoustically solid convex mass with a lower silhouette (ie, lower height-to-base ratios than malignant melanoma). Fluorescein angiography of the mass usually shows early hypofluorescence with no vasculature and late diffuse hyperfluorescence, which, however, is not pathognomonic.

Treatment options

External beam radiotherapy is the standard treatment for choroidal metastasis, with most studies showing an 80% response rate. A larger area of the orbit is treated to address possible metastasis focality. Plaque radiotherapy, on the other hand, is a localized therapy that decreases radiation exposure to surrounding structures. It can be used as primary treatment or in external beam radiotherapy failure. However, once a patient receives plaque therapy, no additional treatment can be given. Although the use of chemotherapy for choroidal metastases is still not widely reported, studies have shown regression of masses in patients who receive chemotherapy for their primary malignancy. These patients need close monitoring for progression.

Figure 5. Funduscopic image of right eye 1 month after treatment with systemic gemcitabine and cisplatin. Pigmentary changes seen over the amelanotic mass.

Our patient was treated with systemic gemcitabine and cisplatin. One month later, best corrected visual acuity in the right eye improved from 20/80 to 20/50. Repeat dilated funduscopic examination showed pigmentary changes associated with regression of choroidal lesions (Figure 5). A repeat B-scan showed the choroidal lesion measuring 1.65 mm high (Figure 6). OCT of the fovea showed reduced subretinal fluid with central foveal thickness of 325 µm (Figure 7).

Figure 6. B-scan of right eye 1 month after chemotherapy. Solid mass elevated by 1.65 mm to 1.7 mm is seen in the posterior pole.

Figure 7. OCT of the right eye 1 month after chemotherapy. Subfoveal fluid is still present but significantly less than before treatment. Central foveal thickness has decreased from 724 µm to 325 µm.

Summary

The diagnosis of choroidal metastases is primarily based on clinical findings and patient history. B-scans and fluorescein angiography of the mass can provide additional data. OCT of the macula can be helpful in revealing subretinal fluid if patients have decreased visual acuity. While external beam radiotherapy is the standard treatment, chemotherapy can be considered as the initial treatment, with good response reported. These patients require close monitoring and additional treatment if there is no evidence of regression or if there are signs of progression.

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• Laurel N. Vuong, MD, and Jay S. Duker, MD, can be reached at New England Eye Center, Tufts University School of Medicine, 750 Washington St., Box 450, Boston, MA 02111; 617-636-4219; fax: 617-636-4866; website: www.neec.com.

• Edited by Priti Batta, MD, and Namrata Nandakumar, MD. Drs. Batta and Nandakumar can be reached at New England Eye Center, Tufts University School of Medicine, 750 Washington St., Box 450, Boston, MA 02111; 617-636-4219; fax: 617-636-4866; website: www.neec.com.