Uveal melanoma: management and outcome of patients with extraocular spread

Abstract

BACKGROUND Extraocular spread is thought to be a negative prognostic factor on the survival in patients with uveal melanoma. Enucleation was the standard treatment in these patients. Today, depending on the size of the tumour and the type of extraocular extension, eye-preserving irradiation treatments, such as proton beams or radioactive plaques, may be employed. METHODS 2256 patients were treated between 2000 and 2007 at the Institut Curie, Paris, France for a uveal melanoma. 67 patients (3.0%) presented an extraocular extension. A retrospective study was performed to evaluate the patients' outcomes with regard to tumour recurrence and survival. RESULTS Eye-conserving treatment was employed in 38 (52.8%) patients. Enucleation was performed in 29 (47.2%) patients. The median follow-up was 38 (range 7 to 79) months with an overall survival rate at 5 years of 40.4% in enucleated patients and 79.3% in the eye-conserving treatment group (protons n=19, iodine-125 plaque n=19) (p=0.01; Kaplan-Meier analysis). No tumour recurrence was observed in any group. The degree of extraocular spread as well as the clinical characteristics tumour location, retinal detachment, ciliary body involvement (p<0.01; chi(2) test) and tumour thickness (p=0.04; chi(2) test) influenced the choice of treatment. Age, tumour diameter, involvement of optic nerve, vitreous haemorrhage and the amount of pigment did not have any influence. CONCLUSIONS Tumour recurrence rates and survival rates were not adversely affected in patients receiving conservative eye treatment. This may thus represent a therapeutic option in certain patients with extraocular spread.

Small Choroidal Melanoma Revealed by a Large Extrascleral Extension

“…In most cases, choroidal melanomas are confined within the scleral envelope. Extraocular extension is present at the time of diagnosis in only 3-5.8% of the patients with uveal melanoma [2,3,4]. It is more common in medium or large size tumors and in tumors located in the ciliary body, at the site of vortex veins and in the juxtapapillary area.…”

“…Primary enucleation is indicated for large tumors (largest basal diameter >16 mm and/or tumor thickness >10 mm), whereas globe-sparing treatments are possible for smaller tumors [3]. There is no consensus on the treatment of small choroidal melanomas with a large extraocular extension.…”

Trial Watch

“…Conversely, brachytherapy relies on the seeding of tiny radioactive pellets within the tumor mass (interstitial brachytherapy), in a adjacent cavity - be it surgical or natural - via needles or catheters (intracavitary brachytherapy), while systemic radiation therapy is based on the oral or intravenous administration of a radionuclide, frequently coupled to a tumor-targeting monoclonal antibody 7 , 8 . A peculiar type of intracavitary brachytherapy is represented by plaque radiotherapy, which is frequently employed for the management of uveal and choroidal melanoma 9 - 11 . This approach involves a thin, concave metal plate containing radioactive seeds (often of 125 I) that is sewn to the outer surface of the ocular globe.…”

“…This approach involves a thin, concave metal plate containing radioactive seeds (often of 125 I) that is sewn to the outer surface of the ocular globe. For how it is constructed (with radioactive seeds facing inward), this medical device allows for the relatively focused delivery of ionizing radiation to the eye (and hence the tumor) while protecting other tissues from exposure 9 - 11 …”

Orbital Extension of an Unsuspected Choroidal Melanoma Presumably through an Aqueous Tube Shunt

“…Extraocular tumor extension is reported to occur in 3-14.6% of eyes enucleated for uveal melanoma [1,2]. Massive orbital invasion is rare and has been diagnosed in 0.5% of patients in a large series [3].…”