engleski

Uveal melanoma: management and prognosis

Educational topic: Uveal melanoma is the most common primary intraocular malignancy in adults and the eye is the second most common site for primary melanoma after the skin. Early recognition is important in protecting visual acuity, saving the eye and preventing metastasis. Discussion: Signs for early detection of uveal melanoma when it simulates a nevus include thickness >2 mm, presence of subretinal fluid, symptoms, orange pigment, margin of the tumour near the optic disc, acoustic hollowness, surrounding halo, and the absence of drusen. This is essential considering that each millimetre increase in melanoma thickness imparts a 5% increased risk for metastatic disease. Delays or inability to make an accurate and early diagnosis may have grave consequences. Methods of diagnosis have substantially improved, although clinical diagnosis remains the standard method in the eyes with clear media. In eyes with opaque media ultrasound is the most useful ancillary diagnostic technique. Newer imaging modalities such as optical coherence tomography and fundus autoflouroscence facilitate in detection of subretinal fluid and orange pigment. Additional molecular biomarkers and cytological features which can predict the clinical behaviour of a small melanocytic lesion have been identified. Advances in the diagnosis and local and systemic treatment of uveal melanoma in recent times have caused a shift from enucleation to eye-conserving treatment modalities. Currently irradiation is the most common therapeutic choice with plaque brachytherapy being the most frequently used form. Other techniques include charged-particle radiotherapy, proton beam therapy and surgery. With a primary tumour, local treatment methods are effective at preventing local recurrence in over 95% of cases. However, they have no impact on the risk of metastatic disease, which develops in up to 50% of patients with spread of the tumour cells most commonly to the liver. Although potential therapeutic targets have been identified there is currently no effective treatment of metastatic disease with systemic therapy and chemotherapy generally being ineffective. Conclusions: Pending clinical trials involving chemotherapeutic, immunotherapeutic and molecularly targeted agents offer hope for successful tumour control and vision preservation as well as metastases prevention and improvement of overall uveal melanoma patient survival.

uveal melanoma ; management ; prognosis

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