Cancer Metastatic to the Orbit : The 2000 Robert M. Curts Lecture

The purpose of this investigation is to report the clinical features of patients with uveal metastases seen at a major ocular oncology center. A retrospective chart review was performed on all patients with uveal metastases evaluated at an ocular oncology outpatient facility over a 20-year period. To assess the systemic and ophthalmic features of uveal metastases. A total of 950 uveal metastases were diagnosed in 520 eyes of 420 consecutive patients. Of the 950 metastatic foci, the uveal involvement included iris in 90 (9%), ciliary body in 22 (2%), and choroid in 838 (88%). The total number of uveal metastases per eye was 1 (71%) in 370 eyes, 2 (12%) in 63 eyes, and 3 or more (17%) in 87 eyes. The mean number of uveal metastases per eye was two (median, one). Iris metastases presented most often as a yellow-to-white solitary nodule in the inferior quadrant. Ciliary body metastases typically presented as a solitary, sessile, or dome-shaped yellow mass in the inferior quadrant, but were difficult to visualize directly. The choroidal metastases typically were yellow in color, plateau shaped, and associated with subretinal fluid. In the 479 eyes with choroidal metastases, the epicenter of the main tumor was found in the macular area in 59 eyes (12%), between the macula and equator in 383 eyes (80%), and anterior to the equator in 37 eyes (8%). The mean size of the main (largest) choroidal tumor in each eye was 9 mm in base and 3 mm in thickness. At the time of ocular diagnosis, 278 patients (66%) reported a history of a primary cancer and 142 patients (34%) had no history of a cancer. Subsequent evaluation of these 142 patients after the ocular diagnosis of uveal metastasis showed a primary tumor in the lung in 50 patients (35%), breast in 10 (7%), others in 9 (6%), and no primary site was found in 73 patients (51%). Nearly half of the patients with no known primary site eventually died of diffuse metastatic disease. In the entire group of 420 patients, the uveal metastasis came from a primary cancer of the breast in 196 (47%), lung in 90 (21%), gastrointestinal tract in 18 (4%), kidney in 9 (2%), skin in 9 (2%), prostate in 9 (2%), and other cancers in 16 (4%). In 73 cases (17%), the primary site was never established despite systemic evaluation by medical oncologists. Iris, ciliary body, and choroidal metastases have typical clinical features that should suggest the diagnosis. The choroid is the most common site for uveal metastases, and the tumors occur most often in the posterior pole of the eye with an average of two tumors per eye. Approximately one third of patients have no history of primary cancer at the time of ocular diagnosis. Breast and lung cancers represent more than two thirds of the primary tumor sites.

The authors reviewed the clinical and histopathologic records of 38 patients with metastatic orbital tumors. Diplopia, ocular motility limitation, and mass effect with displacement, proptosis, or palpable mass were common signs and symptoms. Enophthalmos occurred in 25% of cases. The authors found that the clinical presentations could be broken down into four generalized syndromes of presentation: infiltrative (20 cases, 53%); mass (14 cases, 37%); inflammatory (2 cases, 5%); and functional (1 case, 3%). An infiltrative pattern of presentation may be a clue to the metastatic nature of the orbital tumor. There may be no history of systemic cancer; in 25% of the patients in this series, the orbital tumor was the initial manifestation of systemic disease. Although the prognosis is poor for patients with metastatic cancer (average survival in this series, 10.2 months), modern treatment methods continue to improve and long-term palliation is often possible. The ophthalmologist plays a vital role in the diagnosis, histologic evaluation, and referral of these patients.