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家族性黑素细胞性病变的表型和皮肤镜特征:在三级中心的一项初步研究

Phenotypic and Dermoscopic Patterns of Familial Melanocytic Lesions: A Pilot Study in a Third-Level Center.

作者信息

Roccuzzo Gabriele, Giordano Silvia, Granato Thomas, Cavallo Francesco, Mastorino Luca, Avallone Gianluca, Pasini Barbara, Quaglino Pietro, Ribero Simone

机构信息

Department of Medical Sciences, Section of Dermatology, University of Turin, 10126 Turin, Italy.

Medical Genetics Unit, AOU 'Città Della Salute e Della Scienza'-'Molinette' Hospital, 10126 Turin, Italy.

出版信息

Cancers (Basel). 2023 Jul 25;15(15):3772. doi: 10.3390/cancers15153772.

Abstract

Cutaneous melanoma is a highly aggressive skin cancer. It is estimated that 5% to 10% of the underlying mutations are hereditary and responsible for familial (or hereditary) melanoma. These patients are prone to the early development and higher risk of multiple melanomas. In recent years, an increasing number of genes have been identified thanks to genetic testing, allowing the subsequent surveillance of individuals at risk, yet it is still difficult to predict the presence of these mutations on a clinical basis. In this scenario, specific phenotypic and dermoscopic features could help clinicians in their identification. The aim of this work has been to correlate mutations to prevalent dermoscopic patterns, paving the way for reference models useful in clinical practice. In our cohort, out of 115 patients referred to genetic counseling for melanoma, 25 tested positive (21.7%) for critical mutations: CDKN2A (n = 12), MITF (n = 3), BAP1 (n = 1), MC1R (n = 3), PTEN (n = 1), TYR (n = 2), OCA2 (n = 1), and SLC45A2 (n = 2). The phenotype profiles obtained through the digital acquisition, analysis, and description of both benign and malignant pigmented lesions showed a predominance of the type II skin phenotype, with an elevated mean total nevus number (182 moles, range 75-390). As for dermoscopic features, specific mutation-related patterns were described in terms of pigmentation, areas of regression, and vascular structures. Although further studies with larger cohorts are needed, our work represents the beginning of a new approach to the study and diagnosis of familial melanoma, underlining the importance of clinical and dermoscopic patterns, which may constitute a reference model for each gene, enabling comparison.

摘要

皮肤黑色素瘤是一种侵袭性很强的皮肤癌。据估计,5%至10%的潜在突变是遗传性的,是家族性(或遗传性)黑色素瘤的病因。这些患者易于早期发病且患多发性黑色素瘤的风险更高。近年来,借助基因检测发现了越来越多的基因,从而能够对有风险的个体进行后续监测,但在临床基础上预测这些突变的存在仍然困难。在这种情况下,特定的表型和皮肤镜特征可以帮助临床医生进行识别。这项工作的目的是将突变与常见的皮肤镜模式相关联,为临床实践中有用的参考模型铺平道路。在我们的队列中,115名因黑色素瘤接受遗传咨询的患者中,25人(21.7%)检测出关键突变呈阳性:细胞周期蛋白依赖性激酶抑制剂2A(CDKN2A,n = 12)、小眼畸形相关转录因子(MITF,n = 3)、BRCA1相关蛋白1(BAP1,n = 1)、黑素皮质素受体1(MC1R,n = 3)、第10号染色体缺失的磷酸酶和张力蛋白同源物(PTEN,n = 1)、酪氨酸酶(TYR,n = 2)、眼皮肤白化病II型(OCA2,n = 1)和溶质载体家族45成员2(SLC45A2,n = 2)。通过对良性和恶性色素性病变进行数字采集、分析和描述所获得的表型特征显示,II型皮肤表型占主导,平均痣总数较高(182颗痣,范围75 - 390颗)。至于皮肤镜特征,从色素沉着、消退区域和血管结构方面描述了与特定突变相关的模式。尽管需要更大队列的进一步研究,但我们的工作代表了家族性黑色素瘤研究和诊断新方法的开端,强调了临床和皮肤镜模式的重要性,这可能构成每个基因的参考模型,便于进行比较。

https://cdn.ncbi.nlm.nih.gov/pmc/blobs/191a/10416987/1978a03e075b/cancers-15-03772-g001.jpg

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