Fusion Oncogenes in Salivary Gland Tumors: Molecular and Clinical Consequences

  • G. S
  • Stenman G
  • 1


    Mendeley users who have this article in their library.
  • N/A


    Citations of this article.


Salivary gland tumors constitute a heterogeneous group of uncommon diseases that pose significant diagnostic and therapeutic challenges. However, the recent discovery of a translocation-generated gene fusion network in salivary gland carcinomas as well in benign salivary gland tumors opens up new avenues for improved diagnosis, prognostication, and development of specific targeted therapies. The gene fusions encode novel fusion oncoproteins or ectopically expressed normal or truncated oncoproteins. The major targets of the translocations are transcriptional coactivators, tyrosine kinase receptors, and transcription factors involved in growth factor signaling and cell cycle regulation. Notably, several of these targets or pathways activated by these targets are druggable. Examples of clinically significant gene fusions in salivary gland cancers are the MYB-NFIB fusion specific for adenoid cystic carcinoma, the CRTC1-MAML2 fusion typical of low/intermediate-grade mucoepidermoid carcinoma, and the recently identified ETV6-NTRK3 fusion in mammary analogue secretory carcinoma. Similarly, gene fusions involving the PLAG1 and HMGA2 oncogenes are specific for benign pleomorphic adenomas. Continued studies of the molecular consequences of these fusion oncoproteins and their down-stream targets will ultimately lead to the identification of novel driver genes in salivary gland neoplasms and will also form the basis for the development of new therapeutic strategies for salivary gland cancers and, perhaps, other neoplasms. © 2013 Springer Science+Business Media New York.

Author-supplied keywords

  • Adenoid Cystic
  • Adenoma
  • Carcinoma
  • Fusion
  • Humans
  • Mucoepidermoid
  • Oncogene Proteins
  • Pleomorphic
  • Salivary Gland Neoplasms
  • adenoid cystic carcinoma
  • adenosquamous carcinoma
  • article
  • genetics
  • human
  • oncoprotein
  • pathology
  • pleomorphic adenoma
  • salivary gland tumor

Get free article suggestions today

Mendeley saves you time finding and organizing research

Sign up here
Already have an account ?Sign in

Find this document


  • Stenman G.

  • Goran Stenman

  • Stenman G.

Cite this document

Choose a citation style from the tabs below

Save time finding and organizing research with Mendeley

Sign up for free