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Oncotarget. 2015 Nov 10;6(35):37117-34. doi: 10.18632/oncotarget.5076.

Adenoid cystic carcinoma: current therapy and potential therapeutic advances based on genomic profiling.

Chae YK1,2,3, Chung SY1, Davis AA3, Carneiro BA1,2,3, Chandra S1,2,3, Kaplan J1,2,3, Kalyan A1,2,3, Giles FJ1,2,3.

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Northwestern Medicine Developmental Therapeutics Institute, Northwestern University Feinberg School of Medicine, Chicago, IL, USA.
Robert H. Lurie Comprehensive Cancer Center of Northwestern University, Northwestern University Feinberg School of Medicine, Chicago, IL, USA.
Department of Medicine, Northwestern University Feinberg School of Medicine, Chicago, IL, USA.


Adenoid cystic carcinoma (ACC) is a rare cancer with high potential for recurrence and metastasis. Efficacy of current treatment options, particularly for advanced disease, is very limited. Recent whole genome and exome sequencing has dramatically improved our understanding of ACC pathogenesis. A balanced translocation resulting in the MYB-NFIB fusion gene appears to be a fundamental signature of ACC. In addition, sequencing has identified a number of other driver genes mutated in downstream pathways common to other well-studied cancers. Overexpression of oncogenic proteins involved in cell growth, adhesion, cell cycle regulation, and angiogenesis are also present in ACC. Collectively, studies have identified genes and proteins for targeted, mechanism-based, therapies based on tumor phenotypes, as opposed to nonspecific cytotoxic agents. In addition, although few studies in ACC currently exist, immunotherapy may also hold promise. Better genetic understanding will enable treatment with novel targeted agents and initial exploration of immune-based therapies with the goal of improving outcomes for patients with ACC.


adenoid cystic carcinoma; genetics; immunotherapy; targeted therapy

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