Singh Manni, Durairaj Priya, Yeung Jensen
University of Toronto, Toronto, ON, Canada.
Department of Ocular Oncology, The Princess Margaret Hospital, Toronto, ON, Canada.
Oncol Ther. 2018 Jun;6(1):87-104. doi: 10.1007/s40487-018-0056-8. Epub 2018 Feb 6.
Melanomas affecting different components of the uvea occur with differing frequencies and clinical presentations. Uveal melanoma is diagnosed via funduscopic exam and ancillary tests. These lesions may present with visual findings or incidental findings on physical exam. Metastasis occurs in approximately half of all patients with primary uveal melanoma. The liver is the most common site of metastasis. Enucleation was at one time considered the definitive local treatment for primary uveal melanoma, but has been largely replaced by other therapeutic procedures that aim to prevent metastasis while preserving vision. Unfortunately, metastasis of uveal melanoma almost always proves to be fatal. The current treatment of metastatic uveal melanoma is limited by the intrinsic resistance of uveal melanoma to conventional systemic therapies. Advancements in molecular biology have resulted in the identification of a number of promising prognostic and therapeutic targets. Early detection and therapy are important factors in disease survival. It is imperative that the treating physician be familiar with the clinical features of uveal melanoma and distinguish it from mimickers in order to ensure effective and timely treatment.
影响葡萄膜不同组成部分的黑色素瘤,其发生频率和临床表现各异。葡萄膜黑色素瘤通过眼底检查和辅助检查来诊断。这些病变可能在体格检查时表现为视觉症状或偶然发现。大约一半的原发性葡萄膜黑色素瘤患者会发生转移。肝脏是最常见的转移部位。眼球摘除术曾一度被认为是原发性葡萄膜黑色素瘤的确定性局部治疗方法,但如今在很大程度上已被其他旨在预防转移同时保留视力的治疗方法所取代。不幸的是,葡萄膜黑色素瘤的转移几乎总是致命的。目前转移性葡萄膜黑色素瘤的治疗受到葡萄膜黑色素瘤对传统全身治疗的固有耐药性的限制。分子生物学的进展已导致确定了一些有前景的预后和治疗靶点。早期检测和治疗是疾病生存的重要因素。治疗医生必须熟悉葡萄膜黑色素瘤的临床特征,并将其与相似疾病区分开来,以确保有效及时的治疗。