Choroidal (uveal) melanoma

What is uveal melanoma?

Uveal melanoma is a rare tumour, but the most frequent primary malignant tumour of the eye in adults, with 500 to 600 new cases per year in France. The main risk is the onset of hepatic metastases, other metastatic locations being rarer or occurring later. It may occur de novo or through degeneration of a choroidal naevus. This is why patients with a large choroidal naevus usually benefit from early detection and regular monitoring of the optical fundus.

Melanoma of the eye may be discovered during a routine examination of the fundus or via the occurrence of recent visual problems such as lowering of visual acuity, a spot in the visual field (scotoma), flashes of light, or visual field loss (retinal detachment).

Tumours posterior to or “straddling the equator” can be treated with proton therapy: under general anaesthetic in the operating theatre, the surgeon identifies the tumour and inserts tantalum clips which will serve to guide the proton beam. Whenever possible, a puncture is made with a fine needle during the operation to better characterise the tumour biologically. An endoresection (surgical ablation of the tumour scar tissue) is sometimes carried out after proton therapy to avoid complications, in particular neovascular glaucoma. For small anterior tumours, brachytherapy treatment with iodine 125 may be proposed. These radiotherapy treatments control the tumour locally in 95% of cases. Prolonged monitoring of the ocular scar is essential. Complications (cataracts, glaucoma, radiation retinopathy) are possible and require ophthalmological treatment.

Enucleation or surgical ablation of the eye is reserved for the largest tumours. An extended assessment is necessary to search for distant metastases, particularly involving the liver.

Conjunctival, palpebral and orbital tumours
These tumours are usually treated with a combination of surgery and radiotherapy. Here again, they benefit from a multidisciplinary approach and recent biological advances.

Care management at Institut Curie

Institut Curie has coordinated the MELACHONAT national network under the aegis of INCa since 2013. Its purpose is to enable every patient to access the expert centres for uveal melanoma in France to benefit from the best treatment of this rare tumour. An ophthalmological and oncological multidisciplinary consultation meeting (RCP) dedicated to uveal melanoma has been in place at Institut Curie since 2009.

The other missions of the network are to define national recommendations on best clinical practices, to promote recourse and access to innovation by developing clinical trials within the network, to collect data in a dedicated, computerised national database, to promote research on this rare tumour, to inform the public and train professionals, and to build a healthcare network.

Our research on uveal melanoma

As a national centre of reference for the treatment of choroidal melanoma, a rare eye cancer, Institut Curie is continuing its commitment to better characterize this disease. Yhere has been close collaboration with researchers at Institut Curie for over 10 years regarding uveal melanoma. This has already enabled us to characterize the genetic anomalies of uveal melanoma, to correlate them to the risk of metastasis and to offer the patients concerned a clinical research protocol of adjuvant chemotherapy (FOTEADJ). Several clinical studies are also in progress for treating metastases, in particular early international trials testing new anticancer compounds. Scientific work is continuing in order to understand the disease better and find new, more active medicines (targeted therapies) for melanoma of the eye. In 2016 It starts a research program, a PIC3i, financed by its own funds and to a large extent by the generosity of the general public. The goal is to decode a new method of tumor dissemination.