Uveal melanoma: relatively rare but deadly cancer
Uveal melanoma: relatively rare but deadly cancer
复制标题
DOI：
10.1038/eye.2016.275
复制
发表时间：
2017-02-01
期刊：
影响因子：
3.9
通讯作者：
Shields, C. L.
中科院分区：
文献类型：
--
作者：
Kaliki, S.;Shields, C. L.
虽然葡萄膜黑色素瘤是一种相对罕见的疾病，主要在高加索人群中发现，但葡萄膜黑色素瘤是成年人最常见的原发眼内肿瘤，年龄调整后的平均发病率为每年5.1例/百万例。肿瘤位于虹膜(4%)、睫状体(6%)或脉络膜(90%)。葡萄膜黑色素瘤的宿主易感因素包括皮肤白皙、眼睛颜色浅、不能晒黑、眼或眼皮黑素细胞增多症、皮肤或虹膜或脉络膜痣，以及BRCA1相关蛋白1突变。目前，对这种恶性肿瘤最广泛使用的一线治疗方案是切除、放射治疗和眼球摘除。放射治疗主要有两种类型：斑块近距离放射治疗(碘-125、Ru-106或钯-103或钴-60)和远程治疗(使用数码刀、伽玛刀或直线加速器的质子束、氦离子或立体定向放射外科手术)。替代放射治疗的方法是摘除眼球。虽然这些治疗方法取得了令人满意的局部疾病控制，但葡萄膜黑色素瘤患者的长期存活率仍然很低，有肝转移的风险。在过去的几年里，早期诊断方面取得了进展，人们希望随着较小的肿瘤得到治疗，存活率可以提高。与许多其他癌症适应症一样，早期发现和早期治疗对于葡萄膜黑色素瘤的积极长期生存结果至关重要。这些观察结果提醒人们注意尚未得到满足的医疗需求，即早期治疗眼睛中的小黑色素细胞病变或小黑色素瘤，以实现局部疾病控制和视力保护，并有可能防止转移和提高总体患者存活率。
Although it is a relatively rare disease, primarily found in the Caucasian population, uveal melanoma is the most common primary intraocular tumor in adults with a mean age-adjusted incidence of 5.1 cases per million per year. Tumors are located either in iris (4%), ciliary body (6%), or choroid (90%). The host susceptibility factors for uveal melanoma include fair skin, light eye color, inability to tan, ocular or oculodermal melanocytosis, cutaneous or iris or choroidal nevus, and BRCA1-associated protein 1 mutation. Currently, the most widely used first-line treatment options for this malignancy are resection, radiation therapy, and enucleation. There are two main types of radiation therapy: plaque brachytherapy (iodine-125, ruthenium-106, or palladium-103, or cobalt-60) and teletherapy (proton beam, helium ion, or stereotactic radiosurgery using cyber knife, gamma knife, or linear accelerator). The alternative to radiation is enucleation. Although these therapies achieve satisfactory local disease control, long-term survival rate for patients with uveal melanoma remains guarded, with risk for liver metastasis. There have been advances in early diagnosis over the past few years, and with the hope survival rates could improve as smaller tumors are treated. As in many other cancer indications, both early detection and early treatment could be critical for a positive long-term survival outcome in uveal melanoma. These observations call attention to an unmet medical need for the early treatment of small melanocytic lesions or small melanomas in the eye to achieve local disease control and vision preservation with the possibility to prevent metastases and improve overall patient survival.