O'Neill Iain D
De l'immeuble 3, Centre d'Affaires Poincaré, 3 Rue Poincaré, 06000 Nice, France.
Oral Oncol. 2009 Jan;45(1):2-9. doi: 10.1016/j.oraloncology.2008.03.012. Epub 2008 May 16.
Mucoepidermoid carcinoma (MEC) is a relatively uncommon carcinoma of variable histology that can involve many tissue types, most commonly major and minor salivary glands and the tracheo-bronchial tree. In a significant number of cases a recurring t(11;19) translocation with an associated novel fusion oncogene (CRTC1-MAML2) is present. This translocation is also found in Warthin's tumour and clear cell hidradenoma of the skin. The CRTC1-MAML2 oncogene acts as a transcription factor on Notch and CREB regulatory pathways, disrupting normal cell-cycle and differentiation, contributing to tumour development. Data suggest that in MEC, the presence of CRTC1-MAML2 may have some prognostic value. An understanding of these mechanisms extends our knowledge of the role of fusion oncogenes in epithelial malignancy. A review of CRTC1-MAML2 in MEC is presented.
黏液表皮样癌（MEC）是一种相对不常见的具有多种组织学类型的癌症，可累及多种组织类型，最常见于大、小唾液腺以及气管支气管树。在相当数量的病例中，存在复发性t(11;19)易位以及相关的新型融合癌基因（CRTC1-MAML2）。这种易位也见于沃辛瘤和皮肤透明细胞汗腺瘤。CRTC1-MAML2癌基因作为Notch和CREB调节途径的转录因子，破坏正常细胞周期和分化，促进肿瘤发展。数据表明，在黏液表皮样癌中，CRTC1-MAML2的存在可能具有一定的预后价值。对这些机制的理解扩展了我们对融合癌基因在上皮恶性肿瘤中作用的认识。本文对黏液表皮样癌中的CRTC1-MAML2进行综述。
