(approximately 20% and 35%, respectively)

histologically confirmed metastases at 5 years and 10 years found in approximately 10% and 18% of patients, respectively, in both cohorts

secondary finding in enucleated eyes:

—only 2/660 enucleated eyes misdiagnosed as having a choroidal melanoma secondary findings in patients undergoing brachytherapy:

—10% local tumor recurrence at 5 years

—13% risk of enucleation after brachytherapy at 5 years

—local tumor recurrence weakly associated with a reduced survival

—decline in visual acuity to 20/200 in approximately 40% of patients at 3 years

—quadrupling of the visual angle (6 lines of visual loss) in approximately 50% of patients at 3 years

COMS Small Choroidal Tumor Trial

observational study of 204 patients with tumors measuring 4.0–8.0 mm in basal diameter and/or 1.0–2.4 mm in apical height

melanoma-specific mortality 1% at 5 years

clinical growth factors included

—greater initial thickness and basal diameter —presence of orange pigmentation

—absence of drusen and/or retinal pigment epithelial changes —presence of tumor pinpoint hyperfluorescence on angiography

Detailed findings of the COMS can be found at www.jhu.edu/wctb/coms.

Pigmented Epithelial Tumors of the Uvea and Retina

Adenoma and Adenocarcinoma

Figure 17-15 Peripapillary combined hamartoma of the retina and RPE. A, Fundus photograph showing obscuration of the retinal vessels in superior aspect of the optic disc, pigmentation, retinal striae (arrow), and hard exudates. B, Fluorescein angiogram (19.7 sec) showing the vascular component of this lesion composed of small capillary-like telangiectatic vessels. Note the relative hypofluorescence superior to the optic disc due to the RPE component of this lesion. C, Late fluorescein angiogram (12 min) showing diffuse late fluorescein leakage in the distribution of the lesion. (Courtesy of Robert H. Rosa, Jr, MD.)

Benign adenomas of the nonpigmented and pigmented ciliary epithelium may appear indistinguishable clinically from melanomas arising in the ciliary body. Benign adenomas of the RPE are very rare. These lesions occur as oval, deeply melanotic tumors arising abruptly from the RPE. Adenomas rarely enlarge and seldom undergo malignant change. Adenocarcinomas of the RPE are also very rare; only a few cases have been reported in the literature. Although these lesions have malignant features histologically, their metastatic potential appears to be minimal.

Fuchs adenoma (pseudoadenomatous hyperplasia) is usually an incidental finding at autopsy and rarely becomes apparent clinically. It appears as a glistening, white, irregular tumor arising from a ciliary crest. Histologically, it consists of benign proliferation of the nonpigmented ciliary epithelium with accumulation of basement membrane–like material.

Acquired Hyperplasia

Hyperplasia of the pigmented ciliary epithelium or the RPE usually occurs in response to trauma, inflammation, or other ocular insults. Ciliary body lesions, because of their location, often do not become evident clinically. Occasionally, however, they may reach a large size and simulate a ciliary body melanoma. Posteriorly located lesions may be more commonly recognized and can lead to diagnostic uncertainty. In the early management of these atypical lesions, observation is often appropriate to document stability of the lesion. Adenomatous hyperplasia, which has been reported only rarely, may clinically mimic a choroidal melanoma.

Combined Hamartoma

Combined hamartoma of the RPE and retina is a rare disorder that occurs most frequently at the disc margin. Typically, it appears as a darkly pigmented, minimally elevated lesion with retinal traction and tortuous retinal vessels (Fig 17-15). Glial cells within this lesion may contract, producing the traction lines seen clinically in the retina. Exudative complications associated with the vascular component of the lesion may be observed. This lesion has been mistaken for melanoma because of its dark pigmentation and slight elevation. In rare cases, a combined hamartoma may be situated in the peripheral fundus.

Shields CL, Thangappan A, Hartzell K, Valente P, Pirondini C, Shields JA. Combined hamartoma of the retina and retinal pigment epithelium in 77 consecutive patients’ visual outcome based on macular versus extramacular tumor location. Ophthalmology. 2008;115(12):2246–2252.