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December 2015

New Research Findings

ACCRF's mission is to accelerate the development of improved therapies and a cure for ACC patients. We support the efforts of the ACC research community in:
  • Accessing specimens, models and data
  • Understanding the basic biology of the disease
  • Identifying and developing promising treatments and diagnostics
  • Opening high quality clinical trials
Each step is an essential building block upon which later progress is made. This update highlights some recent developments that demonstrate the vibrant nature of ACCRF's research network. 

Stay tuned for more exciting "ACComplishments"!
Consolidated Genomic Data
ACCRF has updated its Databases webpage to include extensive new information on ACC genomic studies. These files will be of interest primarily to researchers. However, patients with a technical bent may find the resources to be useful as well.
Three sequencing studies - from Memorial Sloan Kettering, the Wellcome Trust Sanger Institute and MD Anderson, and Foundation Medicine - have been published on 112 ACC patients. The lists of somatic mutations from the articles' appendices have been consolidated into one spreadsheet file. In addition, gene expression and copy number alteration data sets are available from a range of studies.
Patient-derived xenograft (PDX) models of ACC have been an invaluable resource for the research community. They are being used to develop cell lines, study the disease biology and screen drugs. We have gathered new and existing data sets that characterize the ACC PDX models so the research community may understand them better and use them even more effectively. 
New Research Findings
Earlier this month, two concurrent papers from Drs. Adel El-Naggar (MD Anderson) and Scott Ness (University of New Mexico) identified a new genetic alteration in ACC tumors. Rearranged chromosomes were found to create MYBL1 gene fusions in approximately 15% of cases. MYBL1 is very closely related to the MYB gene that is rearranged in approximately 70% of cases. It is becoming increasingly clear that similar biological processes underlie the vast majority of ACC cases. Hopefully, these commonalities will make it simpler to discover and develop treatments that will benefit most or all ACC patients. These two papers are just the latest among an impressive list of publications from ACCRF grantees.

Adel El-Naggar
Dr. Adel El-Naggar
Dr. Scott Ness

Another important recent finding relates to the frequency and significance of mutations in the NOTCH1 gene among ACC tumors. Researchers from MD Anderson presented a poster detailing that approximately 14% of ACC patients have activating NOTCH1 mutations and that their overall survival is significantly shorter than for patients without such mutations. In addition, an ACC patient with a NOTCH1 mutation had significant tumor shrinkage after taking a drug targeting NOTCH1. It is important for physicians to identify aggressive cases early so they may monitor such patients more closely and provide appropriate treatments when available. Ongoing research on NOTCH1 at MD Anderson and the Dana-Farber Cancer Institute will help bring such necessary care closer to ACC patients. 

As our researchers understand better the biology of ACC, the chances of finding better treatments rises!
Clinical Trials Update

During 2015, four new articles were published on phase II clinical trials in ACC, covering studies of dovitinib, sorafenib, dasatinib and nelfinavir. And two new posters were presented on phase II clinical trials in ACC, covering studies of dovitinib and MK-2206. Links to each of these reports are available on ACCRF's  Past Studies webpage.

Educating patients and their physicians about completed and promising clinical trials leads to better knowledge and treatment plans.
ACC clinical trials typically are only for patients with progressive disease. Recently, the phase II clinical trial in ACC of regorafenib completed enrollment. Currently, there are no studies of systemic therapies (drugs) still enrolling only ACC patients. However, there are three phase II clinical trials open for salivary gland cancer patients, including ACC patients. These studies are listed on ACCRF's  Current Studies webpage. We anticipate that three or more new ACC-specific clinical trials will open during 2016. ACCRF will inform patients of new studies as soon as possible through this email distribution list and on our website.
  Patients with progressive disease may consider tumor profiling to identify genetic alterations in their particular tumor that have matched targeted therapies. If a tumor profile identifies a NOTCH1 mutation, the patient may consider the NOTCH1 inhibitor clinical trials we list at the bottom of the Current Studies webpage. We also list phase I clinical trials that may be of interest to ACC patients.

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