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Uveal melanoma.
Nature Reviews Disease Primers ( IF 76.9 ) Pub Date : 2020-04-09 , DOI: 10.1038/s41572-020-0158-0
Martine J Jager 1 , Carol L Shields 2 , Colleen M Cebulla 3 , Mohamed H Abdel-Rahman 3, 4 , Hans E Grossniklaus 5, 6 , Marc-Henri Stern 7 , Richard D Carvajal 8 , Rubens N Belfort 9 , Renbing Jia 10 , Jerry A Shields 2 , Bertil E Damato 11
Nature Reviews Disease Primers ( IF 76.9 ) Pub Date : 2020-04-09 , DOI: 10.1038/s41572-020-0158-0
Martine J Jager 1 , Carol L Shields 2 , Colleen M Cebulla 3 , Mohamed H Abdel-Rahman 3, 4 , Hans E Grossniklaus 5, 6 , Marc-Henri Stern 7 , Richard D Carvajal 8 , Rubens N Belfort 9 , Renbing Jia 10 , Jerry A Shields 2 , Bertil E Damato 11
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Uveal melanoma (UM) is the most common primary intraocular malignancy in adults. UMs are usually initiated by a mutation in GNAQ or GNA11, unlike cutaneous melanomas, which usually harbour a BRAF or NRAS mutation. The annual incidence in Europe and the USA is ~6 per million population per year. Risk factors include fair skin, light-coloured eyes, congenital ocular melanocytosis, ocular melanocytoma and the BAP1-tumour predisposition syndrome. Ocular treatment aims at preserving the eye and useful vision and, if possible, preventing metastases. Enucleation has largely been superseded by various forms of radiotherapy, phototherapy and local tumour resection, often administered in combination. Ocular outcomes are best with small tumours not extending close to the optic disc and/or fovea. Almost 50% of patients develop metastatic disease, which usually involves the liver, and is usually fatal within 1 year. Although UM metastases are less responsive than cutaneous melanoma to chemotherapy or immune checkpoint inhibitors, encouraging results have been reported with partial hepatectomy for solitary metastases, with percutaneous hepatic perfusion with melphalan or with tebentafusp. Better insight into tumour immunology and metabolism may lead to new treatments.
中文翻译:
葡萄膜黑色素瘤。
葡萄膜黑色素瘤(UM)是成人最常见的原发性眼内恶性肿瘤。UM 通常由 GNAQ 或 GNA11 中的突变引发,这与皮肤黑色素瘤不同,皮肤黑色素瘤通常具有 BRAF 或 NRAS 突变。欧洲和美国的年发病率约为每年每百万人口 6 人。风险因素包括白皙的皮肤、浅色眼睛、先天性眼部黑素细胞增多症、眼部黑素细胞瘤和 BAP1 肿瘤易感性综合征。眼部治疗旨在保护眼睛和有用的视力,并在可能的情况下预防转移。摘除术在很大程度上已被各种形式的放射疗法、光疗法和局部肿瘤切除术所取代,这些疗法通常结合使用。对于不靠近视盘和/或中央凹的小肿瘤,眼部结果最好。近 50% 的患者发生转移性疾病,这通常涉及肝脏,通常在 1 年内致命。尽管 UM 转移瘤对化疗或免疫检查点抑制剂的反应不如皮肤黑色素瘤,但对于孤立性转移瘤的部分肝切除术、经皮肝灌注美法仑或替苯他福已报告了令人鼓舞的结果。更好地了解肿瘤免疫学和代谢可能会带来新的治疗方法。
更新日期:2020-04-09
中文翻译:
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葡萄膜黑色素瘤。
葡萄膜黑色素瘤(UM)是成人最常见的原发性眼内恶性肿瘤。UM 通常由 GNAQ 或 GNA11 中的突变引发,这与皮肤黑色素瘤不同,皮肤黑色素瘤通常具有 BRAF 或 NRAS 突变。欧洲和美国的年发病率约为每年每百万人口 6 人。风险因素包括白皙的皮肤、浅色眼睛、先天性眼部黑素细胞增多症、眼部黑素细胞瘤和 BAP1 肿瘤易感性综合征。眼部治疗旨在保护眼睛和有用的视力,并在可能的情况下预防转移。摘除术在很大程度上已被各种形式的放射疗法、光疗法和局部肿瘤切除术所取代,这些疗法通常结合使用。对于不靠近视盘和/或中央凹的小肿瘤,眼部结果最好。近 50% 的患者发生转移性疾病,这通常涉及肝脏,通常在 1 年内致命。尽管 UM 转移瘤对化疗或免疫检查点抑制剂的反应不如皮肤黑色素瘤,但对于孤立性转移瘤的部分肝切除术、经皮肝灌注美法仑或替苯他福已报告了令人鼓舞的结果。更好地了解肿瘤免疫学和代谢可能会带来新的治疗方法。