Uveal Melanoma; Current Trends in The Diagnosis and Management

Abstract

Uveal melanoma, which is the most common primary intraocular malignancy in adults, arises from melanocytes within the iris, ciliary body and choroid. The diagnosis is based principally on clinical examination of the tumor with biomicroscopy and indirect ophthalmoscopy and confirmed by diagnostic techniques such as ultrasonography, fundus fluorescein angiography and optical coherence tomography. The clinical diagnosis of posterior uveal melanomas can be made when the classical appearance of a pigmented dome-shaped mass is detected on dilated fundus exam. Uveal melanomas classically show low to medium reflectivity on A-scan ultrasonography and on B-scan ultrasonography the tumor appears as a hyperechoic, acoustically hollow intraocular mass. Management of a suspicious pigmented lesion is determined by its risk factors of transforming into a choroidal melanoma, such as documentation of growth, thickness greater than 2 mm, presence of subretinal fluid, symptoms and orange pigment, margin within 3 mm of the optic disc, and absence of halo and drusen. Advances in the diagnosis and local and systemic treatment of uveal melanoma have caused a shift from enucleation to eye-conserving treatment modalities including transpupillary thermotherapy and radiotherapy over the past few decades. Prognosis can be most accurately predicted by genetic profiling of fine needle aspiration biopsy of the tumor before the treatment, and high-risk patients can now be identified for clinical trials that may lead to target-based therapies for metastatic disease and adjuvant therapy which aims to prevent metastatic disease.

Malpractice Litigation in Ocular Oncology

“…A European study found similar incidence results that varied with latitude from a low of <2 per million in Spain and southern Italy to a high of >8 per million in Norway and Denmark [24]. Uveal melanoma accounts for 85% of ocular melanoma and is the most common primary intraocular malignancy among adults [22,23,25,26]. The majority of uveal melanoma originates in the choroid (90%), while origin in the ciliary body (7%) or iris (2%) is less common [25,26].…”

“…Uveal melanoma accounts for 85% of ocular melanoma and is the most common primary intraocular malignancy among adults [22,23,25,26]. The majority of uveal melanoma originates in the choroid (90%), while origin in the ciliary body (7%) or iris (2%) is less common [25,26]. Prognosis depends on a number of factors, including age and sex, tumor diameter, ciliary body involvement, and cytomorphology and genetics [27].…”

Recent advances in uveal melanoma treatment

“…In addition to genetic factors, morphologic characteristics also impact prognosis: tumor thickness greater than 2 mm, presence of subretinal fluid and orange pigment, tumor margin within 3 mm of the optic disc, ultrasonographic hollowness, and absence of halo are high‐risk predictive factors …”

“…76 In addition to genetic factors, morphologic characteristics also impact prognosis: tumor thickness greater than 2 mm, presence of subretinal fluid and orange pigment, tumor margin within 3 mm of the optic disc, ultrasonographic hollowness, and absence of halo are high-risk predictive factors. 78 There are several characteristics that increase the probability of UM development. Age, gender, and ethnicity are related to different incidence rates of the disease: UM risk increases with age and peaks at the age of 70 years; males develop UM more frequently than females and Caucasians are more likely to develop UM compared to populations…”

Orbital recurrence of iris melanoma 21 years after enucleation

“…Among all uveal melanomas, those which originate from the iris manifest the less aggressive behaviour 6. Nonetheless, they must be recognised and treated early since they can progress to invade adjacent structures and lead to metastatic disease 7. Differentiation from other benign iris lesions, such as nevi and cysts, relies mainly on clinical grounds: documented substantial growth over time remains the main predictive feature of malignancy 8.…”