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

Young Kwang Chae _, Su Yun Chung, Andrew A Davis, Benedito A Carneiro, Sunandana Chandra, Jason Kaplan, Aparna Kaylan and Francis J Giles

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Oncotarget. 2015; 6:37117-37134. https://doi.org/10.18632/oncotarget.5076

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Young Kwang Chae1,2,3, Su Yun Chung1, Andrew A. Davis3, Benedito A. Carneiro1,2,3, Sunandana Chandra1,2,3, Jason Kaplan1,2,3, Aparna Kalyan1,2,3, Francis J. Giles1,2,3

1Northwestern Medicine Developmental Therapeutics Institute, Northwestern University Feinberg School of Medicine, Chicago, IL, USA

2Robert H. Lurie Comprehensive Cancer Center of Northwestern University, Northwestern University Feinberg School of Medicine, Chicago, IL, USA

3Department of Medicine, Northwestern University Feinberg School of Medicine, Chicago, IL, USA

Correspondence to:

Young Kwang Chae, e-mail: [email protected]

Keywords: adenoid cystic carcinoma, targeted therapy, genetics, immunotherapy

Received: June 05, 2015     Accepted: August 08, 2015     Published: August 21, 2015


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.

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