1] Division of Molecular Genetic Epidemiology, German Cancer Research Center, 69120 Heidelberg, Germany [2].
1] Department of Dermatology, Instituto Valenciano de Oncologia, Valencia 46009, Spain [2] Universidad Catolica de Valencia, Valencia 46001, Spain [3].
Nat Commun. 2014 Feb 26;5:3401. doi: 10.1038/ncomms4401.
We previously reported a disease segregating causal germline mutation in a melanoma family and recurrent somatic mutations in metastasized tumours from unrelated patients in the core promoter region of the telomerase reverse transcriptase (TERT) gene. Here we show that the TERT promoter mutations, besides causing an increased gene expression, associate with increased patient age, increased Breslow thickness and tumour ulceration in 287 primary melanomas. The mutations are more frequent at both intermittently and chronically sun-exposed sites than non-exposed sites and tend to co-occur with BRAF and CDKN2A alterations. The association with parameters generally connected with poor outcome, coupled with high recurrence and mechanistic relevance, raises the possibility of the eventual use of TERT promoter mutations in the disease management.
我们之前报道了一个黑色素瘤家族中的疾病分离性种系突变,以及在无关联的转移性肿瘤中核心启动子区域的端粒酶逆转录酶(TERT)基因的反复出现的体细胞突变。在这里,我们表明 TERT 启动子突变除了导致基因表达增加外,还与 287 例原发性黑色素瘤中的患者年龄增加、Breslow 厚度增加和肿瘤溃疡有关。这些突变在间歇性和慢性暴露于阳光的部位比非暴露部位更为频繁,并且往往与 BRAF 和 CDKN2A 改变同时发生。与通常与不良预后相关的参数的关联,加上高复发率和机制相关性,增加了 TERT 启动子突变在疾病管理中的最终应用的可能性。