Woman presents with severe pain, elevated IOP in right eye

On examination, lesions were found in both the right and left eyes.

Priti Batta

Namrata Nandakumar

A 53-year-old Caucasian woman was referred to the New England Eye Center with severe eye pain and a conjunctival mass. She was being treated for iritis by an outside ophthalmologist and was noted to have iris and conjunctival lesions in the right eye. On presentation, she reported having blurry vision in her right eye for 1 month, severe eye pain, photophobia and foreign body sensation.

Her ocular history was significant for myopia and presbyopia. Her medical history was significant for stage 2 breast adenocarcinoma that was diagnosed in 2009. She successfully underwent a lumpectomy, chemotherapy and radiation, and the breast cancer was thought to be in remission. Her current medications included Pred Forte (prednisolone acetate ophthalmic suspension, Allergan) every hour in the right eye, homatropine three times per day in the right eye, Motrin (ibuprofen, McNeil Consumer Healthcare) and Percocet (oxycodone hydrochloride and acetaminophen, Endo Pharmaceuticals) for pain.

On exam, the patient’s best corrected visual acuity was counting fingers at 2 feet in the right eye and 20/25 in the left eye. The right pupil was dilated, and the left pupil was reactive, with no reverse afferent pupillary defect. IOP by Goldmann applanation tonometry was 38 mm Hg in the right eye and 9 mm Hg in the left eye. Extraocular motility was full in both eyes.

Anterior segment exam of the right eye was significant for a flesh-colored iris lesion nasally and a nodular 3-mm perilimbal conjunctival mass at the nasal limbus with surrounding injection (Figure 1). Gonioscopy revealed extension of the mass into the angle, with complete absence of normal angle anatomy for 3 to 4 contiguous clock-hours. There was also microcystic edema of the cornea, 3+ flare and no cells in the anterior chamber, and 1+ nuclear sclerotic cataract. Ultrasound biomicroscopy demonstrated a hyperreflective ciliary body mass, extending into the iris and the conjunctiva (Figure 2). Anterior segment exam of the left eye was relatively unremarkable, including a white and quiet conjunctiva, clear cornea, deep and quiet anterior chamber, and 1+ nuclear sclerotic cataract.

Figure 1. Anterior segment photos of the right eye are notable for a flesh-colored iris lesion and a nodular conjunctival lesion. Images: Williams SL, Hu DJ

Figure 2. Ultrasound biomicroscopy demonstrates a hyperreflective ciliary body mass, with extension into the iris and conjunctiva.

Figure 3. Fundus photos of the left eye demonstrates a 5-mm, cream-white colored choroidal mass in the superior midperiphery.

Figure 4. Ultrasound demonstrates an elevated choroidal lesion with low internal reflectivity.

Posterior segment exam of the right eye was limited by dense vitritis, but no retinal lesions were evident. The posterior segment exam of the left eye showed clear media, sharp disc margins and a normal-appearing posterior pole. There was a 5-mm creamy-white, elevated, choroidal mass in the superior mid-periphery (Figure 3). Ultrasound demonstrated a subretinal mass of low echogenicity (Figure 4). There were no associated hemorrhages, subretinal fluid in the macula or other retinal lesions.

What is your diagnosis?

Bilateral lesions

The differential diagnosis of the anterior segment lesions includes primary malignancies of the conjunctiva and iris, such as amelanotic nodular melanoma, squamous cell carcinoma, medulloepithelioma, as well as lymphoid tumors. The rapid growth of the lesion could be consistent with an inflammatory or infectious cause, such as sarcoid or tuberculosis. Metastatic lesions should also be considered. The contralateral choroidal lesion is suspicious for an amelanotic choroidal melanoma, an inflammatory process or a metastatic lesion. Given the patient’s history and exam findings, the most likely diagnosis is ocular metastasis from her breast cancer.

Discussion

Metastasis to the eye and adnexa occurs in 4% to 12% of all cancer patients. The most common primary tumors are breast cancer in women and lung cancer in men, although ocular metastasis occurs with many other cancers, including lymphoma/leukemia and gastrointestinal, prostate, kidney and thyroid cancers. The most common site of ocular metastasis is the uvea (predominantly to the choroid), followed by the orbit. Conjunctival metastasis is extremely rare but has been reported, with cutaneous melanoma, breast and lung cancers being the most common primary cancers. Patients can also have multiple sites of ocular metastasis.

Among cases of breast cancer with uveal metastases, many are bilateral, often with one eye being completely asymptomatic. Although most patients complain of blurry vision, most present with visual acuity of better than 20/40. Other common symptoms include floaters and photopsias. Elevation of IOP is uncommon but can occur in the setting of anterior segment inflammation and compromise of the trabecular meshwork.

Diagnosis and management

Ocular metastasis should be suspected among patients with a known history of cancer as well as patients who are elderly, are immunocompromised, or have a family history of cancer or other cancer risk factors. In addition to a complete ocular examination, ocular imaging with ultrasound, fluorescein angiography and optical coherence tomography can be used to delineate the size and extent of the metastasis, as well as to monitor response to treatment. Because patients with ocular metastases often have other systemic sites of involvement, imaging with CT or MRI is also critical. Incisional or excisional biopsies can be done when possible to confirm a diagnosis and to help guide treatment. The mainstays of treatment of metastatic breast cancer are external beam radiation, chemotherapy and plaque radiation. Complications from treatment are rare but include radiation retinopathy, papillopathy and neovascular glaucoma. Although many patients undergo regression of the ocular lesions, the overall prognosis for metastatic disease is poor.

Figure 5. Biopsy of the bulbar conjunctiva and limbus was stained with H&E and estrogen receptor immunohistochemical stains, respectively, and demonstrated adenocarcinoma consistent with metastatic breast cancer. Tissue was also estrogen receptor positive, but progesterone receptor and HER2/neu receptor were negative. Image: Laver N

Our patient was started on IOP-lowering medications. She also underwent an incisional biopsy of the conjunctiva, which confirmed a diagnosis of metastatic breast adenocarcinoma (Figure 5). Additional imaging confirmed brain and lung metastases. She underwent treatment with external beam radiation and chemotherapy, which resulted in partial regression of the ocular metastases.

References:

• Demirci H, Shields CL, Chao AN, Shields JA. Uveal metastasis from breast cancer in 264 patients. Am J Ophthalmol. 2003;136(2):264-271.
• Jakobiec FA, Buckman G, Zimmerman LE, et al. Metastatic melanoma within and to the conjunctiva. Ophthalmology. 1989;96(7):999-1005.
• Kiratli H, Shields CL, Shields JA, DePotter P. Metastatic tumours to the conjunctiva: report of 10 cases. Br J Ophthalmol. 1996;80(1):5-8.
• Shields CL, Shields JA, Gross NE, Schwartz GP, Lally SE. Survey of 520 eyes with uveal metastases. Ophthalmology. 1997;104(8):1265-1276.
• Yanoff M, Duker JS. Ophthalmology. 3rd ed. Philadelphia: Elsevier; 2009.

• Steven L. Williams, MD, and Daniel J. Hu, 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.