Title

ENUCLEATION TO TREAT UVEAL MELANOMA: STILL A REALITY IN DEVELOPING COUNTRIES?

Introduction

Uveal Melanomas are the most common form of primary intraocular malignancy, with an estimated worldwide incidence of 6 per million. Radiotherapy is now the treatment of choice in cases of uveal melanomas but enucleation still is required in many patients. Here we present a case of ciliary body melanoma manifesting as unilateral ocular cataract that went undetected in the preoperative evaluation, beeing refered to our retinal ambulatory after surgery procedure and treated by enucleation.

Methods

Case report and review of the relevant literature about uveal melanomas.

Results

Ciliary body(CB) melanomas represents about 6% of all Uveal Melanomas, and because of their position, they normally go undetected until they are in an advanced stages.Their position makes them extremely difficult to notice through an undilated pupil so they can be easily ignored in a routine ophthalmic examination and due to that when the diagnosis occurs radiotherapy cannot be performed.

Discussion

Despite successful eradication of the ocular tumor, up to 50% of patients develop metastatic disease, which typically involves the liver and is usually fatal within a year of onset of symptoms. Factors predicting metastasis include older age, large tumor size, ciliary body involvement, extraocular spread, epithelioid cytomorphology, chromosome 3 loss, chromosome 8q gain, class 2 gene expression profile, loss of BRCA1-associated protein-1 (BAP1), and the presence of inflammation. Many of them were present in our case. Although radiotherapy is now the first choice in cases of uveal melanomas, enucleation still is required many times. Anatomical and functional globe preservation must be the main objectives, and so, radiotherapy should always be tried when possible, however, due to the difficulty in assisting and monitoring patients who live in poorly developed distant cities and lack of radiotherapy centers, enucleation becomes the only therapeutic option and unfortunately a reality in developing countries.

Keywords

Uveal Melanoma; Ciliary Body Melanoma.

Area

CLINICAL CASE

Authors

RAPHAEL SANTANA MIRALDI CLEMENTE, MARINA COSTA LACERDA, REBECA GRANGEIRO de CARVALHO CABRAL, BERNARDO ANTONIO NEGREIROS MOTA, JOSE FABIO de OLIVEIRA MIRANDA, CARLOS AUGUSTO MOREIRA NETO, CARLOS AGUSUTO MOREIRA JUNIOR, LEANDRO SELESTRINO VERONEIS