Agaimy A, Weichert W, Haller F, Hartmann A
Institut für Pathologie, Universitätsklinikum Erlangen, Friedrich-Alexander-Universität Erlangen-Nürnberg, Krankenhausstraße 8-10, 91054, Erlangen, Deutschland.
Institut für Pathologie, Technische Universität, München, Deutschland.
Pathologe. 2018 Feb;39(1):42-47. doi: 10.1007/s00292-018-0416-z.
As a result of some seminal observations as well as a consequence of increasing use of modern and innovative molecular diagnostic technologies, a variety of new genetic aberrations have been discovered in head and neck neoplasms of different anatomic locations and histogenetic origins. These advances resulted in the establishment of new molecularly defined disease entities. On the other hand, some of these new genetic biomarkers paved the way to potentially promising novel therapeutic opportunities. Diverse old (well known in other entities) and newly discovered translocations and gene fusions represent the leading subgroup of these genetic aberrations. They have been detected not only in malignant epithelial neoplasms (carcinomas) of the salivary glands, but also in carcinomas from other head and neck sites as well as diverse mesenchymal tumors. In addition to these gene fusions, several activating mutations (such as CTNNB1 in sinonasal glomangiopericytoma) as well as inactivating mutations or deletions (like SMARCB1 loss in sinonasal carcinomas) were detected as new molecular markers. In the present review we summarize the relevant molecular alterations in topographically and histopathologically distinct tumors of the head and neck region with emphasis on recently established molecular markers.
由于一些开创性的观察结果以及现代创新分子诊断技术使用的增加,在不同解剖位置和组织发生学起源的头颈部肿瘤中发现了多种新的基因畸变。这些进展导致了新的分子定义疾病实体的建立。另一方面,其中一些新的基因生物标志物为潜在的有前景的新型治疗机会铺平了道路。各种已知的(在其他实体中常见)和新发现的易位及基因融合是这些基因畸变的主要亚组。它们不仅在唾液腺的恶性上皮性肿瘤(癌)中被检测到,也在其他头颈部部位的癌以及各种间叶性肿瘤中被检测到。除了这些基因融合外,还检测到了一些激活突变(如鼻窦血管外皮细胞瘤中的CTNNB1)以及失活突变或缺失(如鼻窦癌中的SMARCB1缺失)作为新的分子标志物。在本综述中,我们总结了头颈部区域在地形学和组织病理学上不同的肿瘤中的相关分子改变,重点是最近建立的分子标志物。