t(11;19) translocation and CRTC1-MAML2 fusion oncogene in mucoepidermoid carcinoma

Oral Oncol. 2009 Jan;45(1):2-9. doi: 10.1016/j.oraloncology.2008.03.012. Epub 2008 May 16.

Abstract

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.

Publication types

  • Review

MeSH terms

  • Adenolymphoma / genetics
  • Carcinoma, Mucoepidermoid / genetics*
  • Carcinoma, Mucoepidermoid / pathology
  • Cell Cycle / genetics
  • Cell Line, Tumor
  • Chromosomes, Human, Pair 11 / genetics
  • Chromosomes, Human, Pair 19 / genetics
  • DNA-Binding Proteins / genetics
  • Female
  • Gene Expression Regulation, Neoplastic
  • Humans
  • Lung Neoplasms / genetics*
  • Lung Neoplasms / pathology
  • Male
  • Nuclear Proteins / genetics
  • Oncogene Proteins, Fusion / genetics*
  • Salivary Gland Neoplasms / genetics*
  • Salivary Gland Neoplasms / pathology
  • Trans-Activators
  • Transcription Factors / genetics
  • Translocation, Genetic / genetics*

Substances

  • CRTC1 protein, human
  • DNA-Binding Proteins
  • MAML2 protein, human
  • Nuclear Proteins
  • Oncogene Proteins, Fusion
  • Trans-Activators
  • Transcription Factors