Uveal melanoma management is challenging due to its metastatic propensity. DecisionDx-UM is a prospectively validated molecular test that interrogates primary tumor biology to provide objective information about metastatic potential that can be used in determining appropriate patient care. To evaluate the continued clinical validity and utility of DecisionDx-UM, beginning March 2010, 70 patients were enrolled in a prospective, multicenter, IRB-approved study to document patient management differences and clinical outcomes associated with low-risk Class 1 and high-risk Class 2 results indicated by DecisionDx-UM testing. Thirty-seven patients in the prospective study were Class 1 and 33 were Class 2. Class 1 patients had 100% 3-year metastasis-free survival compared to 63% for Class 2 (log rank test p = 0.003) with 27.3 median follow-up months in this interim analysis. Class 2 patients received significantly higher-intensity monitoring and more oncology/clinical trial referrals compared to Class 1 patients (Fisher's exact test p = 2.1 × 10−13 and p = 0.04, resp.). The results of this study provide additional, prospective evidence in an independent cohort of patients that Class 1 and Class 2 patients are managed according to the differential metastatic risk indicated by DecisionDx-UM. The trial is registered with Clinical Application of DecisionDx-UM Gene Expression Assay Results (NCT02376920).
The management of conjunctival surface melanomas is complicated by local recurrence and metastatic disease, particularly for lesions greater than 2 mm in thickness and arising from nonbulbar conjunctiva. Early complete excision with appropriate adjuvant cryotherapy and topical chemotherapy may be curative in some patients. Conjunctival anatomy and high recurrence rate with resultant metastatic spread undermines the utility of sentinel lymph node biopsy in this condition. Further research and innovation for detection of systemic micrometastases and treatment of metastatic disease are needed.
Paraneoplastic retinopathies comprise a diverse group of immune-mediated conditions affecting the eye. Cancer-associated retinopathy (CAR) typically has a sudden onset of severe visual loss and an ominous association with an occult malignancy. CAR is one of the best studied and better understood conditions in the multifarious group of autoimmune retinopathies. Recent developments have correlated the disease presentation, course, and therapeutic response to the underlying immune mediators and the inciting antigens. Signaling involving cytotoxic T-lymphocytes antigen 4 (CTLA-4) and vascular endothelial growth factor (VEGF) receptor-1 appears to play a role in the pathogenesis and may offer novel avenues for therapeutic intervention in CAR. Future developments in rapid identification and longitudinal quantification of antibody levels would enable individualized management in these patients. The goal of this review is to analyze the clinical features of diagnosis and management of retinopathy in the context of recent advances in the elucidation of CAR pathogenesis.
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