Format

Send to

Choose Destination
Head Neck. 2016 Apr;38(4):620-7. doi: 10.1002/hed.23925. Epub 2015 Jun 16.

Adenoid cystic carcinoma: A review of recent advances, molecular targets, and clinical trials.

Author information

1
Division of Hematology/Oncology, University of Virginia, Charlottesville, Virginia.
2
Division of Hematology/Oncology, New Jersey Medical School, Newark, New Jersey.
3
Department of Pathology, University of Virginia, Charlottesville, Virginia.
4
Department of Internal Medicine, University of Virginia, Charlottesville, Virginia.
5
Division of Hematology/Oncology, Wake Forest University, Winston-Salem, North Carolina.

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.

CONCLUSION:

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.

KEYWORDS:

MYB gene; NFIB gene; adenoid cystic carcinoma; nuclear factor I/B; salivary gland; tyrosine kinase inhibitor

PMID:
25487882
PMCID:
PMC6166139
DOI:
10.1002/hed.23925
[Indexed for MEDLINE]
Free PMC Article

Supplemental Content

Full text links

Icon for Wiley Icon for PubMed Central
Loading ...
Support Center