Melanocytomas of the optic disk.

PURPOSE: To evaluate the various forms of clinical presentation, the potential of growth, the risk of functional loss and the possibility of malignant transformation of optic disk melanocytomas in European patients. DESIGN: Retrospective observational case control study. METHODS: Evaluation of 37 cases of optic disk melanocytomas identified in the computer files of the ocular oncology unit of Jules Gonin Hospital to determine the clinical presentation of these tumors and the risk of complications. RESULTS: The tumor was asymptomatic in 28 cases. Visual acuity was normal in 26 cases, subnormal in 10 cases, and in one case it was reduced to 0.1. Visual field defects were observed in 19 cases; the size and extent of the tumor and the degree of papilledema appeared to influence the severity of the visual field defect. Tumor growth was demonstrated in 6 of the 9 cases in which follow-up was for at least six years. In two cases there was presumed malignant transformation that was treated by accelerated proton beam radiotherapy. CONCLUSIONS: Melanocytomas in white Europeans and those of European derivation are rarely symptomatic, have only a moderate effect on visual function and show a low rate of progression over long observation. The risk of tumor progression, although sometimes occurring as much as several years after the initial diagnosis, justifies a cautious approach with long-term regular surveillance of these patients.

Metastatic melanoma in the eye and orbit.

OBJECTIVE: Presentation of a large series of patients with metastatic melanoma involving the eye and orbit. DESIGN: Retrospective clinical study. PARTICIPANTS: Thirteen cases of metastatic melanoma involving intraocular tissues, 6 cases of metastatic melanoma in the orbit, and 1 case of metastatic melanoma involving both the eye and the orbit, treated in Lausanne between 1986 and 2002, were identified from the computer files of the ocular oncology and orbitopalpebral surgery units of Jules Gonin Hospital. METHODS: The analysis is based on the demographic data concerning these patients, clinical data concerning the tumor, the treatment applied, the outcome, and the follow-up. MAIN OUTCOME MEASURES: Clinical presentation of intraocular and orbital metastasis, interval between primary tumor and ocular metastasis, survival of the patients, evaluation of various therapeutic protocols. RESULTS: Intraocular metastases (14 cases, 15 eyes) were situated in the choroid in 11 cases (isolated lesion, 6 cases; multiple lesions, 3 cases; diffuse involvement, 2 cases), in the iris and ciliary body in 2 cases, and in the retina and vitreous in 2 other cases. The primary tumor was a cutaneous melanoma in 8 cases, a melanoma of the contralateral eye in 3 cases, a mucosal melanoma in 1 case, and was unknown in 2 cases. The mean interval between the diagnosis of ocular metastases and the patient's death was 8.8 months (range, 1-48 months). The primary tumor in the 7 cases of orbital metastases was a cutaneous melanoma in 5 cases, a uveal melanoma in the contralateral eye in 1 case, and was unknown in 1 case. The mean interval between the diagnosis of orbital metastases and death was 19.7 months (range, 5-48 months). The patients were treated by various protocols. The best results, in terms of both local tumor control and preservation of visual function, were obtained with circumscribed proton beam radiotherapy or external beam irradiation, depending on the site and extent of the tumor. CONCLUSIONS: Metastatic melanomas to the eye and orbit are rare and generally occur in patients with disseminated metastases during the terminal stages of the disease, with a short life expectancy. Treatment is palliative and, among the various possible treatment options, circumscribed proton beam radiotherapy or global photon beam radiotherapy, at relatively high irradiation doses, seems to achieve the most favorable results.