title: Adenoid cystic carcinoma: A review of recent advances, molecular targets, and clinical trials.
abstract: BACKGROUND
  Adenoid cystic carcinoma (ACC) is a rare tumor of secretory glands. In this study, recent advances in molecular characterization and in therapeutics are reviewed.
  
  
  METHODS
  A search of articles in PubMed and of abstracts from national meetings was performed regarding ACC.
  
  
  RESULTS
  Recent genetic analyses found that recurrent chromosome 6:9 translocations in ACC generate an MYB:NFIB gene fusion resulting in overexpression of the MYB oncoprotein. Several other frequent mutations are recently published that may be relevant for drug development. Several trials of targeted drugs are reviewed. Some agents delay tumor progression, but tumor responses remain rare.
  
  
  CONCLUSIONS
  ACCs have a characteristic chromosomal translocation, but also frequently pick up additional mutations. Clinical research is limited by the rarity and slow growth of ACC. Several ongoing trials are testing agents that inhibit fibroblast growth factor receptor signaling or other signaling pathways. Novel treatments based on the recently sequenced tumor genome are under development.
authors.affiliation: Division of Hematology/Oncology, University of Virginia, Charlottesville, Virginia.
authors.forename: Patrick M
authors.initials: PM
authors.lastname: Dillon
authors.affiliation: Division of Hematology/Oncology, New Jersey Medical School, Newark, New Jersey.
authors.forename: Samhita
authors.initials: S
authors.lastname: Chakraborty
authors.affiliation: Department of Pathology, University of Virginia, Charlottesville, Virginia.
authors.forename: Christopher A
authors.initials: CA
authors.lastname: Moskaluk
authors.affiliation: Department of Internal Medicine, University of Virginia, Charlottesville, Virginia.
authors.forename: Prashant J
authors.initials: PJ
authors.lastname: Joshi
authors.affiliation: Division of Hematology/Oncology, Wake Forest University, Winston-Salem, North Carolina.
authors.forename: Christopher Y
authors.initials: CY
authors.lastname: Thomas
mesh_terms: D003528:Carcinoma, Adenoid Cystic / Q000235:genetics* / Q000473:pathology / Q000628:therapy; D002896:Chromosomes, Human, Pair 6; D002899:Chromosomes, Human, Pair 9; D002986:Clinical Trials as Topic; D005260:Female; D020570:Genes, myb / Q000235:genetics; D020022:Genetic Predisposition to Disease; D006801:Humans; D008297:Male; D058990:Molecular Targeted Therapy / Q000379:methods*; D009154:Mutation; D012468:Salivary Gland Neoplasms / Q000235:genetics* / Q000473:pathology / Q000628:therapy; D014178:Translocation, Genetic
keywords: MYB gene; NFIB gene; adenoid cystic carcinoma; nuclear factor I/B; salivary gland; tyrosine kinase inhibitor