Uveal melanoma (UM) is the most common primary malignant intraocular tumor in adults, with a 10-year cumulative metastatic rate of 34%. The most common site of metastasis is the liver (95%). Unfortunately, the current treatment of metastatic UM is limited by the lack of effective systemic therapy. Options for the management of the primary intraocular tumor include radical surgery as well as conservative treatments in order to preserve visual acuity. For metastatic disease, several approaches have been described with no standard method. Nevertheless, median survival after liver metastasis is poor, being around 4–6 months, with a 1-year survival of 10%–15%. In this review, the authors summarize current and promising new treatments for UM.
RESUMOA síndrome de frouxidão palpebral é caracterizada pela fácil eversão da pálpebra que ocorre espontaneamente durante o sono causando exposição dos olhos, com consequente conjuntivite papilar crônica. Sua patogênese ainda não está totalmente definida e é mais comum em pacientes masculinos, obesos, de meia idade e está associada a doenças sistêmicas como apnéia obstrutiva do sono, hipertensão arterial e diabetes. Quando o tratamento clínico não apresenta boa resposta, procedimentos cirúrgicos apresentam bons resultados, com técnicas em constante evolução.Descritores: Conjuntiva/patologia; Conjuntivite; Ectrópio; Obesidade/complicações; Hipertensão/complicações; Diabete Mellitus/complicações; Apnéia do sono tipo obstrutiva/complicações; Doenças palpebrais/cirurgia; Síndrome; Músculos oculomotores/cirurgia; Pálpebras/cirurgia; Doenças da córnea ABSTRACTFloppy eyelid syndrome is characterized by the easy evertion of the upper eyelid which occurs spontaneously during the sleep, causing the exposure of the eye surface and chronic papillary conjunctivitis. Its pathogenesis is not totally defined yet: it is usually more frequent in middle-aged, male obese patients and it is associated with systemic disorders such as obstructive sleep apnea, high blood pressure and diabetes. On the occasions which conservative treatment fails, surgical procedures present good results, including surgical techniques which are constantly evolving.
Uveal melanoma is a rare but life-threatening malignancy. Over the past decades, the morbidity of uveal melanoma has been markedly reduced as a result of advances in the diagnostic ability to detect smaller tumors at an earlier stage. This has allowed for the use of more conservative treatments, avoiding enucleation. Mortality, however, has remained unchanged. This indicates that life expectancy is independent of local tumor control. Metastatic disease, the leading cause of death, is usually diagnosed many years later, despite successful treatment of the primary tumor, and at a late stage, when no effective therapy is available. These observations suggest that the disease was already disseminated at the time of tumor diagnosis. The detection of circulating malignant cells in the bloodstream of patients at different time points in the course of the disease supports this observation. Tumor dormancy has been considered as the leading theory for this intriguing delayed appearance of metastasis. Recent knowledge gained about the biological behavior of uveal melanoma as well as novel potential therapeutic targets are presented in this review.
Although rare, uveal melanoma is the most common intraocular tumor in adults. Most cases arise from the choroidal layer of the uvea, displaying a discoid, collar-button, or mushroom shaped growth. Histopathologically, neoplasms are classified by the dominant cell type: spindle, epithelioid or mixed spindle cell type. The most important prognostic factors are cell type, nucleolar size, largest tumor dimension, and mitotic figures. Patient prognosis is poor when metastases occur in the liver, one of the main reasons that despite advances in the diagnosis and treatment of uveal melanoma, the mortality rate has not change significantly since 1973.
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