Hafner Christian, van Oers Johanna M M, Hartmann Arndt, Landthaler Michael, Stoehr Robert, Blaszyk Hagen, Hofstaedter Ferdinand, Zwarthoff Ellen C, Vogt Thomas
Department of Dermatology, University of Regensburg, Regensburg, Germany.
J Invest Dermatol. 2006 Nov;126(11):2404-7. doi: 10.1038/sj.jid.5700422. Epub 2006 Jun 15.
FGFR3 germline mutations cause autosomal dominant skeletal disorders including achondroplasia, thanatophoric dysplasia, severe achondroplasia with developmental delay and acanthosis nigricans, and Crouzon syndrome. Somatic mutations of FGFR3 have been identified in bladder cancer, multiple myeloma, and other neoplasms. FGFR3 mutations have also been detected in 40% of seborrheic keratoses (SKs) of the hyperkeratotic and acanthotic subtype, which are very common benign skin tumors. Using a multiplex SNaPshot assay that covers 11 activating FGFR3 mutations, we investigated a series of 27 SKs of the adenoid subtype. Mutations were detected in 23 of 27 (85%) adenoid SKs. R248C mutations were the most frequent mutation type. In two SKs, the A393E mutation was found, which has not been described in acanthotic and hyperkeratotic SKs so far. Three adenoid SKs displayed two simultaneous FGFR3 mutations. Adenoid SKs seem to be characterized by a higher frequency of FGFR3 mutations than hyperkeratotic and acanthotic SKs. The mechanism for the high rate of somatic FGFR3 mutations in these benign skin tumors remains elusive, but UV light exposure may play a potential role, especially in the R248C mutations.
FGFR3种系突变会导致常染色体显性遗传性骨骼疾病,包括软骨发育不全、致死性骨发育不良、伴有发育迟缓及黑棘皮病的严重软骨发育不全,以及克鲁宗综合征。FGFR3的体细胞突变已在膀胱癌、多发性骨髓瘤及其他肿瘤中被发现。在40%的角化过度型和棘皮瘤型脂溢性角化病(SKs)中也检测到了FGFR3突变,脂溢性角化病是非常常见的良性皮肤肿瘤。我们使用一种覆盖11种激活型FGFR3突变的多重SNaPshot检测法,对一系列27例腺样亚型SKs进行了研究。在27例腺样SKs中有23例(85%)检测到了突变。R248C突变是最常见的突变类型。在两例SKs中发现了A393E突变,该突变迄今为止在棘皮瘤型和角化过度型SKs中尚未见报道。三例腺样SKs表现出两种FGFR3突变同时存在。腺样SKs似乎具有比角化过度型和棘皮瘤型SKs更高频率的FGFR3突变。这些良性皮肤肿瘤中FGFR3体细胞突变率高的机制仍不清楚,但紫外线暴露可能起潜在作用,尤其是在R248C突变中。