Patient complains of acute unilateral photopsias and metamorphopsia

A choroidal lesion and subretinal fluid were seen in the left eye.

Mark E. Patron

Andre J. Witkin

A 53-year-old woman presented to her optometrist with complaints of decreased visual acuity, photopsias and metamorphopsia in her left eye for 2 days and was referred to our eye clinic for ophthalmic consultation.

The patient’s medical history was significant only for remote cesarean section. She had no significant medical history and was not taking medications. She had quit smoking 20 years ago, following a 10 pack-year smoking history. Review of symptoms was notable for dry cough.

Examination

On examination, the patient’s best corrected visual acuity was 20/25 in the right eye and 20/125 in the left. IOPs were 16 mm Hg in the right eye and 18 mm Hg in the left. Anterior segment exam showed trace nuclear sclerosis of both lenses. Pupillary exam was normal with no afferent pupillary defect. Dilated funduscopic examination (Figure 1) of the left eye showed clear media and a healthy optic nerve. The vessels were within normal limits. A 2-mm cream-colored choroidal lesion was present supratemporal to the optic nerve. Subretinal fluid was extending from the lesion into the macula and fovea. B-scan ultrasonography (Figure 2) demonstrated subretinal fluid overlying a lesion with medium internal reflectivity.

Figure 1. Fundus photograph of the left eye taken at initial presentation, showing an elevated cream-colored choroidal mass supratemporal to the optic nerve. Images: Monson BK, Marx J

Figure 2. B-scan demonstrating a 2.4-mm elevation of a choroidal lesion with medium internal reflectivity.

What is your diagnosis?

Choroidal lesion

The differential diagnosis of a patient with a mildly elevated choroidal lesion with subretinal fluid includes choroidal metastasis, amelanotic choroidal melanoma, choroidal hemangioma, primary intraocular lymphoma, posterior scleritis, idiopathic sclerochoroidal calcification, bullous central serous choroidopathy, uveal effusion syndrome, vitelliform dystrophy, Harada’s disease, and tuberculoma or granulomatous inflammation.

Differential diagnosis

Amelanotic choroidal melanoma can closely resemble metastatic carcinomas. Ultrasound can help make the distinction, as metastatic carcinoma usually appears relatively bright (sonoreflective) internally, but amelanotic choroidal melanoma typically appears relatively dark (sonolucent). Choroidal hemangioma more typically presents as an orange-red colored mass. Vitelliform dystrophy and idiopathic sclerochoroidal calcification can closely resemble bilateral metastatic carcinoma to the choroid. Vitelliform lesions can usually be distinguished by fluorescein angiography as they block fluorescence. Idiopathic sclerochoroidal calcifications are characteristically extremely bright (sonoreflective) on ultrasonography, shadowing the orbital soft tissues. Lymphoma typically presents as yellowish-white geographic infiltrates with clumping of the overlying retinal pigment epithelium and occurs bilaterally in 80% to 90% of patients. Posterior scleritis may manifest with chorioretinal changes that consist of subretinal exudates and hemorrhages, but generally have a stippled appearance to the retinal pigment as well. Harada’s disease is characterized by bilateral exudative uveitis, primarily in the posterior segment of the eye, accompanied by pleocytosis of cerebrospinal fluid.

Given the characteristic choroidal appearance and new onset of the lesion, neoplastic growth was suspected. The patient was referred to her primary care physician to rule out neoplastic as well as infectious causes. Chest X-ray revealed a mass in her right lung (Figure 3) prompting a chest CT, which showed a large 4.4 cm × 5.8 cm right lower lobe mass with encased pleural thickening, segmental bronchial and pulmonary artery branch obstruction, mediastinal adenopathy, as well as adrenal gland metastatic foci. T2 MRI of the brain showed a 1 cm × 8 cm bright solid enhancing right frontal lobe lesion. PET scan confirmed uptake in the right lower lung lesion, right infrahilar lymphadenopathy and detected faint metastatic lesions involving thoracic vertebral bodies and left iliac bone as well as a large right adrenal mass, characteristic of primary lung cancer with stage IV metastatic disease. Bronchoscopic biopsy led to a diagnosis of primary adenocarcinoma of the lung.

Figure 3. Posterior-anterior chest X-ray showing a bronchogenic adenocarcinoma in the periphery of the right lung.

Treatment

The patient was subsequently referred to radiation oncology and will be treated with four cycles of systemic cisplatin, pemetrexed and Avastin (bevacizumab, Genentech) chemotherapy. The patient underwent stereotactic radiosurgery for treatment of the brain metastasis. She also received an intravitreal injection of bevacizumab for treatment of the choroidal lesion and has been referred for ocular proton beam irradiation should the bevacizumab prove insufficient.

Discussion

The most common ocular tumor is metastatic carcinoma, which predominantly presents in the choroid. Most patients who present with metastatic carcinoma to the eye have a known diagnosis of cancer. However, in approximately 25% of cases, the metastasis to the eye is the initial finding, leading to the primary diagnosis, as in our patient.

The most common primary sites of metastatic carcinoma to the choroid are the breast in women and the lung in men. Lung cancer accounts for up to 29% of patients presenting initially with symptomatic choroidal metastasis. Choroidal metastasis is generally thought to be a marker of late-stage cancer, and the presence of choroid metastasis from a lung primary is a poor prognostic marker. Mean survival after screening of 84 patients with primary lung cancer with choroidal metastasis in one series was 1.9 months (range: 0.2 to 5.9 months).

Two recent case reports have demonstrated complete resolution of choroidal metastasis secondary to non-small cell lung cancer in response to systemic bevacizumab injection and chemotherapy. Bevacizumab may provide another option in the treatment of choroidal metastasis.

Proton beam irradiation is a highly useful therapeutic approach for choroidal metastatic therapy. Proton beam irradiation allows for retention of the globe and helps to avoid pain and visual loss associated with surgical tumor localization. It has a high success rate with local tumor control, reported at 84%. Although complications occur in 56% of cases, including dry eye syndrome, cataract, neovascular glaucoma, chorioretinal atrophy, radiation papillopathy and radiation maculopathy, most of these side effects are mild and not associated with diminished function.

This case underscores the important role ophthalmologists play in the detection and subsequent treatment of systemic neoplasms. Creamy-yellow choroidal lesions with subretinal fluid and an associated decrease in vision are highly suspicious for choroidal metastases. Extensive investigation for a systemic primary lesion is essential in these patients. If imaging is not productive, local choroidal fine-needle aspiration biopsy may be performed.

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• Bryan K. Monson, MD, and Jeffrey Marx, 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; Web site: www.neec.com.

• Edited by Mark E. Patron, MD, and Andre J. Witkin, MD. Drs. Patron and Witkin 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; Web site: www.neec.com.