Man presents with unilateral vision loss, white spot in eye
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A 73-year-old man presented to the emergency department at Tufts Medical Center with shortness of breath and blurry vision in the right eye for the past 2 months.
The patient reported that his vision had become increasingly blurry over the previous 2 weeks, and upon awakening the morning of presentation, he had lost approximately 90% of vision in the right eye. Additionally, his daughter, who accompanied him to the emergency department, noticed a white spot in his right eye that had been growing in size over the past several weeks.
Source: Jonathan Caranfa, MD, and Melina Morkin, MD
At the time of presentation, the patient denied ocular pain, redness, discharge, flashes or diplopia. Systemic review of systems was notable for shortness of breath and weight loss/anorexia. His medical history was significant for hypertension, hyperlipidemia, chronic obstructive pulmonary disease and stage IV non-small cell lung carcinoma status post radiation and chemotherapy. His ocular and family histories were noncontributory. The patient was a retired farmer, currently married and lived at home with his wife and daughter who provide most of the patient’s activities of daily living. He had no known drug allergies and denied use of alcohol or recreational drugs. The patient noted an approximate 40 pack-year history of tobacco usage.
Examination
Upon examination, visual acuity was 20/800 without correction in the right eye and 20/200 without correction in the left eye. The right pupil was nonreactive, irregular and peaked at 1 o’clock; however, the left pupil was round, brisk and reactive without relative afferent pupillary defect. Extraocular movements were full and painless bilaterally. The patient was unable to participate in visual fields to confrontation and with color plates in the right eye; however, the left eye was full in regard to both examinations. IOP was 35 mm Hg in the right eye and within normal limits in the left eye.
Anterior slit lamp examination of the left eye was unremarkable. Examination of the right eye demonstrated mild conjunctival injection with a large soft tissue mass in the anterior chamber from 2 to 7 o’clock, extending toward and distorting the pupil with pseudohypopyon inferiorly. There was trace cell and multiple inflammatory debris floating in the anterior chamber with keratic precipitates noted inferiorly (Figure 1). Posterior pole exam of both eyes demonstrated pink and healthy-appearing optic nerves with a moderate cup-to-disc ratio. No macular or peripheral retinal abnormalities were found on the dilated exam.
What is your diagnosis?
See answer below.
Soft tissue mass
The differential diagnosis in a patient experiencing progressive vision loss, unilateral ocular hypertension and a soft tissue mass with mild inflammatory response must include infectious, inflammatory/autoimmune and neoplastic processes. In the setting of known carcinoma, metastatic infiltration (solid tumors, lymphoma or leukemia) of the eye is highest on our differential; however, primary tumors such as melanoma, iris pigment epithelial adenocarcinoma, leiomyoma and rhabdomyosarcoma must also be considered.
Infectious etiologies to consider include endophthalmitis (exogenous and endogenous), syphilis, tuberculosis and invasive fungal disease. Inflammatory etiologies, although less likely, include sarcoidosis, HLA-B27-associated disease and juvenile xanthogranuloma.
Workup and management
A complete blood count, QuantiFERON Gold, fluorescent treponemal antibody absorption, angiotensin-converting enzyme, lysozyme and chest X-ray were obtained. To further investigate an underlying systemic malignancy, CT or PET scan of the chest, abdomen and pelvis is often recommended. Finally, ultrasound biomicroscopy (UBM) or anterior segment OCT (AS-OCT) may prove helpful in visualizing and characterizing the anterior chamber lesion. As with almost any lesion, definitive diagnosis is made via biopsy.
Discussion
Of the uveal metastases, choroidal lesions are by far the most common at approximately 80%, with iris metastasis constituting less than 10% of all uveal tumors. The underlying pathophysiology for this discrepancy is believed to be a result of the abundant supply of posterior ciliary arteries to the choroid allowing for greater flow of tumor emboli to the posterior uvea. This is in contrast to the fewer anterior ciliary vessels supplying the anterior uvea. Commonly, uveal metastasis presents in women between the fifth and sixth decade of life with approximately 90% occurring in white patients.
Iris metastasis often presents as a yellow to white solitary nodular lesion found in the inferior quadrant. The most common presenting symptoms are reduced visual acuity, often less than 20/200, and ocular pain as a result of elevated IOP, tumor necrosis, inflammation or scleral involvement. Additionally, a subset of patients will experience new-onset flashes or floaters; however, there remains a significant percentage of patients who will be asymptomatic at the time of diagnosis. Objective findings that may alert ophthalmologists to a potential metastatic process include unexplained or unilateral elevations in IOP, ill-defined iris thickening, hyphema, corectopia, iris neovascularization in the absence of other risk factors, and signs of ocular inflammation such as anterior chamber cell and keratic precipitates. Any unilateral findings should prompt careful examination of the fellow eye as bilateral disease has been reported in up to 20% of patients.
Approximately two-thirds of patients have a known primary cancer diagnosis at the time uveal tumors are discovered while one-third of patients have yet to be diagnosed with a primary cancer. In a survey conducted by Shields and colleagues in 1997 examining 520 eyes with uveal metastases, they demonstrated that in 82% of patients with uveal metastases, the primary cancer was found to be a carcinoma, with melanoma being the second most common primary tumor at 3%. Of the patients with carcinomas, breast and lung were by far the most common at 47% and 21%, respectively.
Diagnosis and treatment
Uveal metastases are diagnosed mainly through direct observation; however, several ophthalmological imaging modalities can help to visualize and better characterize lesions that are suspicious for malignancy. As mentioned previously, both UBM and AS-OCT are useful in visualizing anterior segment lesions and may identify concerning features for malignancy, including tissue depth, extension into surrounding structures and vascularization. Definitive diagnosis can be obtained via fine needle aspiration or incisional/excisional biopsy.
Treatment of anterior uveal metastatic lesions is multimodal and variable depending on the patient’s functional status, extent of ocular involvement and other systemic findings of neoplastic disease. Localized tumors can be treated with either external beam radiation or plaque radiation therapy. In patients with multifocal or bilateral ocular lesions with or without other systemic metastases, chemotherapy can be initiated under the direction of an oncologist. If the patient undergoes radiotherapy, they should be monitored closely for ocular sequelae of radiation exposure including dry eye, cataract formation, glaucoma and radiation retinopathy.
Outcomes in patients with iris metastases are overall poor, with the median time interval from diagnosis to death estimated to be 10 months.
Clinical course continued
During the patient’s emergency department workup and hospitalization, he was found on CT and MRI to have evidence of further metastatic progression of his non-small cell lung carcinoma in association with a new loculated pleural effusion requiring thoracentesis drainage. The patient was started on IOP-lowering agents in his right eye with a stable ocular examination several days after presentation. After extensive discussions with the patient’s family and the oncology team, the decision was made not to pursue aggressive diagnostics and/or treatment. Subsequently, the patient was placed on hospice level care and died shortly thereafter.
- References:
- Shields CL, et al. Arch Ophthalmol. 1997;doi:10.1001/archopht.1997.01100150205010.
- Shields CL, et al. Cornea. 2015;doi:10.1097/ICO.0000000000000285.
- Shields CL, et al. Ophthalmology. 1997;doi:10.1016/s0161-6420(97)30148-1.
- Shields CL, et al. Ophthalmology. 2012;doi:10.1016/j.ophtha.2011.07.059.
- Sobottka B, et al. Br J Ophthalmol. 1998;doi:10.1136/bjo.82.2.159.
- Xiao W, et al. JAMA Ophthalmol. 2019;doi:10.1001/jamaophthalmol.2018.5029.
- For more information:
- Jonathan Caranfa, MD, and Melina Morkin, MD, can be reached at New England Eye Center, Tufts University School of Medicine, 800 Washington St., Box 450, Boston, MA 02111; website: www.neec.com.
- Edited by Yi Ling Dai, MD, and Teresa P. Horan, MD. They can be reached at New England Eye Center, Tufts University School of Medicine, 800 Washington St., Box 450, Boston, MA 02111; website: www.neec.com.