t(11;19) translocation and CRTC1-MAML2 fusion oncogene in mucoepidermoid carcinoma.
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.
O'Neill
Iain D
ID
De l'immeuble 3, Centre d'Affaires Poincaré, 3 Rue Poincaré, 06000 Nice, France. iain.oneill@hotmail.com
D000235:Adenolymphoma / Q000235:genetics; D018277:Carcinoma, Mucoepidermoid / Q000235:genetics* / Q000473:pathology; D002453:Cell Cycle / Q000235:genetics; D045744:Cell Line, Tumor; D002880:Chromosomes, Human, Pair 11 / Q000235:genetics; D002888:Chromosomes, Human, Pair 19 / Q000235:genetics; D004268:DNA-Binding Proteins / Q000235:genetics; D005260:Female; D015972:Gene Expression Regulation, Neoplastic; D006801:Humans; D008175:Lung Neoplasms / Q000235:genetics* / Q000473:pathology; D008297:Male; D009687:Nuclear Proteins / Q000235:genetics; D015514:Oncogene Proteins, Fusion / Q000235:genetics*; D012468:Salivary Gland Neoplasms / Q000235:genetics* / Q000473:pathology; D015534:Trans-Activators; D014157:Transcription Factors / Q000235:genetics; D014178:Translocation, Genetic / Q000235:genetics*
C500984:CRTC1 protein, human; D004268:DNA-Binding Proteins; C471297:MAML2 protein, human; D009687:Nuclear Proteins; D015514:Oncogene Proteins, Fusion; D015534:Trans-Activators; D014157:Transcription Factors