ACCRF Logo Website
June 2014

The continued support of the entire ACC community - both patients and researchers - is making a difference in improving our scientific understanding and treatment options. The ACCRF Scientific Forum at the Salk Institute, recent clinical trial developments, NIH grant announcements and patient events are the latest signs of our shared progress.
ACCRF Scientific Forum at the Salk Institute
ACCRF's recently revised Research Agenda, The Landscape of ACC Research: Past, Ongoing and Future Explorations reviewed the amazing accomplishments of ACCRF's network of investigators and mapped out some priority areas for the coming years. However, the job of improving the foundation's research strategy is never complete. In that spirit, ACCRF convened a gathering of interested researchers on April 5th at the Salk Institute for Biological Studies in La Jolla, CA, graciously hosted by Drs. Irwin Jacobs and Inder Verma. 


The meeting brought together more than 30 researchers, including the ACCRF Scientific Advisory Board, to discuss recent research developments. An impressive group of speakers made presentations as fodder for the lively discussions: 


  • Genomic portraits of ACC - Dr. Tim Chan (Memorial Sloan-Kettering) 
  • The significance of MYB-activation and 1p deletions in ACC - Dr. Göran Stenman (Gothenburg University)
  • Epigenetic states and dependencies in ACC - Dr. Brad Bernstein (Mass General)
  • Transcriptome analysis of ACC salivary gland tumors - Dr. Kathryn Brayer (University of New Mexico)
  • Update from the ongoing screens of investigational and FDA-approved therapies in PD models of ACC - Dr. Michael Wick (START)
  • Early outcomes from a trial of dovitinib for ACC and insights for future trial design - Dr. Patrick Dillon (University of Virginia)
  • Clinical trials for ACC - Dr. Alan Ho (Memorial Sloan-Kettering)
  • Concepts for ACC clinical trials - Dr. John Heymach (MD Anderson)
  • Use of zebrafish to shut off the c-Myb gene - Dr. Len Zon (Boston Children's)
  • Targeted inhibition of Myb function - a novel approach to ACC, T-ALL, and other leukemias - Dr. John Bushweller (University of Virginia)


Understanding the biology and the druggability of the MYB gene was highlighted as a crucial objective. Yet many meeting participants also felt that additional molecular targets might be relevant and impactful in improving patient outcomes. And there was obvious energy among everyone about generating promising, science-driven clinical trial concepts and collaborating to make them happen. In sum, the ACCRF Scientific Forum at the Salk Institute was a galvanizing event that served to strengthen and invigorate the community of researchers in the field of ACC research.


Clinical Trials

ACC patients with progressive disease that cannot be addressed with radiation, surgery or interventional radiology often turn to systemic therapy (targeted drugs and chemotherapy). Given the lack of any approved drugs for ACC, nor a consensus on the most effective drugs, clinical trials of systemic therapy may be appropriate in consultation with a patient's physician. Fortunately, there has been substantial activity in exploring new drugs in ACC in clinical trials.


Report on the Recent Axitinib Study - At the recent Annual Meeting of the American Society of Clinical Oncology (ASCO), a poster presented the preliminary findings of the phase II study of Axitinib in ACC. Axitinib is a drug that targets VEGFR, PDGFR and CKIT. The abstract may be viewed at Of 33 ACC patients with progressive disease, 1 patient had a partial response (substantial tumor shrinkage) and 10 patients had disease stabilization for 6 months or longer. These results are modestly encouraging in the context of past ACC clinical trials:


Closing of the Recent Dovitinib Study - The Canadian phase II study of Dovitinib recently completed enrollment of its 20 ACC patients. Dovitinib is a drug that targets FGFR, VEGFR and PDGFR. Prior phase II studies at the University of Virginia and Seoul National University have been accrued fully, so there are no phase II Dovitinib studies enrolling ACC patients at the moment. Preliminary results from the University of Virginia were reported at last year's ASCO Annual Meeting: The preliminary results were quite encouraging, although further analysis of the data from all three studies will be needed to determine whether additional studies of Dovitinib in ACC will be initiated. 


Opening of the New Regorafenib Study - Memorial Sloan-Kettering recently opened a phase II study of Regorafenib, a drug that targets FGFR, VEGFR and PDGFR (similar to Dovitinib). The estimated enrollment at the New York, NY location will be 32 ACC patients.  


Opening of the New Pembrolizumab (MK-3475) Study - Merck Pharmaceuticals recently opened an arm of a phase IB study of Pembrolizumab (MK-3475) specifically for salivary gland cancer patients (including ACC patients). The drug targets PD-1, an immune checkpoint that can restrain the immune system's attacks on tumors. The study has 18 sites open across the USA, Europe, Canada, Korea and Japan. 


Updated Phase I Studies - Clinical trials that recruit ACC patients in particular may not be convenient or appropriate for all ACC patients. Therefore, ACCRF also lists selected phase I studies on its Current Studies webpage that enroll patients with multiple tumor types. The studies involve drugs that inhibit targets suspected of contributing to the progression of ACC, such as FGFR, IGF-1R and TRK. Two of the studies combine an approved FGFR inhibitor (Pazopanib) with HDAC inhibitors (Vorinostat and Abexinostat), two classes of drugs that have shown activity in significant subsets of ACC patients.   

Options for Patients with NOTCH-Mutated Tumors - Most ACC tumors have similar biology in that the MYB gene is overly active. However, each ACC tumor has its own molecular profile with its own alterations. A small but significant subset of ACC tumors have been found to harbor mutations in NOTCH pathway genes. For patients who have had tumor profiling that identified a NOTCH alteration, ACCRF has listed at the bottom of its Current Studies webpage some phase I studies of NOTCH inhibitors that may be appropriate.  


In all cases, ACC patients who are considering clinical trials should discuss their situations and options with their physicians.


The National Institute of Dental and Craniofacial Research (NIDCR), a component of the National Institutes of Health (NIH), recently announced a request for research proposals to accelerate the development of targeted therapies for oral cancer patients based on genomic studies and bioinformatics. The funding opportunity is entitled "Targeting Co-dependent Molecular Pathways in Oral Cancer (U01)" and is available at


As stated in the description, "The goal of the [announcement] is the identification of co-dependent survival and proliferation pathways in oral cancer cells and the elucidation of potential signaling pathways that can be targeted with combination therapy approaches. This initiative requires collaboration of multi-disciplinary research teams that include cancer biologists, computational biologists, model system specialists and/or screening assay specialists. The emphasis will be on the conceptual phase of target identification and exploration including proof-of-concept phase."


ACC is one of the oral cancers that may be studied. NIDCR intends to commit up to $3 million in fiscal year 2015 to fund 3-4 awards, each lasting for 4 years. Application budgets are limited to $600,000 per year in direct costs. Applications are due by August 25, 2014.


Friends of ACCRF: Patient Gathering & Fundraiser
2014 ACC Survivor Meeting

On March 8th, more than 55 ACC survivors and family members met in Needham, MA to socialize and connect with other ACC families.  We enjoyed learning about each person's hopes and fears and listened to presentations from ACCOI President Tom Curry (to learn about support for the ACC patient community) and ACCRF Executive Director Jeff Kaufman (to learn about current ACC research).  It was a wonderful afternoon spending time with such positive, supportive and very funny new friends!
Drs. Wong, Chan, Liebsch, Bernstein and Haddad enjoy the evening
Later that evening, the 8th Annual Friends of ACCRF event drew a record 260 people and raised over $78,000!  This year's fundraiser attracted scores of patients and ACC researchers who traveled from 11 states to be a part of the special evening. The event featured food, music, an auction and a lot of fun.  Most importantly, every dollar raised went directly to support ACC research.


partnersRunning to Raise Money for ACCRF - June 28, 2014

Live Like Andi Run/Walk
Please join other ACC families and friends at the

Catholic Central High School 
27225 Wixom Road 
Novi, MI 48374

For more information and to register:

 Please Help Support ACC Research