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ACCRF Update
December 2019

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sino2nd Sino-US Symposium on ACC

ACCRF Co-Founders Marnie and Jeff Kaufman recently traveled to Shanghai, China, for the 2nd Sino-US Symposium on ACC, hosted by Dr. Guopei Zhu of the Shanghai Ninth People's Hospital. The  agenda featured topics spanning surgical strategy, biomarkers, targeted therapy, immunotherapy, radiation and clinical trial design. Dr. Alan Ho of Memorial Sloan Kettering Cancer Center also spoke at the symposium and accompanied Marnie and Jeff Kaufman to meetings with some of the 130 Chinese ACC patients attending the associated ACC Clinic.
Chinese researchers have been very active in ACC. Dr. Zhu led the phase II clinical trial of Apatinib. Chinese firms are developing new drugs, such as APG-115, for ACC patients. And Dr. Xiaohong Chen and his colleagues at Beijing Tongren Hospital have been developing new studies. 

Building strong bridges among patients and researchers globally will lead more quickly to better treatments for all ACC patients. 
Genomic Landscape Research
Gathering large numbers of rare cancer cases for research always poses a challenge. However, a broad group of ACCRF-coordinated institutions collaborated to bring together more than 1,000 ACC cases for analysis in a recent publication,  Genetic hallmarks of recurrent/metastatic adenoid cystic carcinoma . Led by Memorial Sloan Kettering, the group effort included Foundation Medicine, MD Anderson, UCLA, UCSF, Chicago, Virginia and Yale. The data is publicly available and analyzable through  cBioPortal .

Among the many fascinating findings, the study identified genes that are mutated at a higher rate in recurrent/metastatic tumors than in primary tumors. The NOTCH genes figure prominently but also several genes involved in chromatin remodeling, such as KDM6A, MLL3 and ARID1B. All will be important topics for understanding the basic biology of ACC as well as potential new therapies.
clinicaltrialsClinical Trials
Since 2005, ACCRF has been supporting basic and translational research with the ultimate goal of getting good scientific ideas into clinical trials. That is the best way for patients to learn whether new therapies truly will help them. Fortunately, the ACC research community has generated a growing list of good clinical trials and will  continue the trend in 2020.
Clinical trials are almost always for patients with advanced and progressing disease (metastases or inoperable primary tumors that are growing). Such patients may keep track of studies that are currently recruiting ACC patients at ACCRF's   Current Studies  webpage. In addition, the  Past Studies  webpage reviews how drugs have performed in previous clinical trials.
Over the past few months, clinical trials of Cabozantinib and the combination of Nivolumab and Ipilumumab have  completed enrollment of patients. And the study of All-Trans Retinoic Acid (ATRA) has  paused enrollment for a few months until preliminary data from the first stage has been assessed. Most importantly, the following studies have  opened :
  • Rivoceranib (also called Apatinib) - Following a promising phase II study carried out in Shanghai, China, several sites will be opening soon in the USA and Korea. For now, the clinical trial is open only at UCSF in San Francisco, CA, USA.
  • Axitinib + Avelumab - Combining immunotherapy with a targeted drug, this study is open only at MD Anderson in Houston, TX, USA.
  • Nivolumab + Ipilumumab + Radiation - Following the completion of two studies of Nivolumab + Ipilumumab, a new study will be testing that immunotherapy combination with radiation. The clinical trial is at the University of Washington in Seattle, WA, USA.
  • AL101 - For ACC patients with activating NOTCH mutations, there are new study sites open throughout Europe, including Denmark, France, Spain and the United Kingdom. Many sites also are open in the USA and Canada.
In September, at the European Society of Medical Oncology annual meeting, there were two interesting preliminary reports from completed clinical trials:
  • GSK3326595 - This study of a PRMT5 inhibitor resulted in 3 partial responses (major tumor shrinkage of 30% or more) among 14 ACC patients, or 21%. Based upon these results, we anticipate the opening of an ACC-focused clinical trial in the coming months.
  • Anlotinib - This inhibitor of VEGFR, FGFR, PDGFR and c-KIT led to partial responses in 19% of salivary gland cancer patients and 79% of patients had not progressed after 6 months. Although there was no separate reporting of activity in ACC patients, they represented 11 of the 21 salivary gland cancer patients.
  ACCRF will continue to keep patients apprised of the latest clinical developments.
tumordonTumor Donations, Cell Lines and the Cancer Dependency Map
Patients can have a direct impact on finding new ways to fight ACC. By donating tumor specimens from upcoming surgeries, patients permit researchers to create new cell lines (human tumors growing in plastic dishes) that help identify disease vulnerabilities and new targets for therapies. At the Broad Institute of MIT and Harvard, the Rare Cancer Dependency Map Initiative is creating ACC cell lines from living tissue and then analyzing them with genomic (CRISPR) and drug screens. The effort is described in this  video.
If you have an upcoming surgery scheduled in the United States, you can help this initiative by donating excess tissue from your procedure. Go to to learn more about tumor donations.
ACCRF is proud to support the work of and the Broad Institute for the benefit of ACC patients.
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Sign up with ACCRF on AmazonSmile and 0.5% of your purchases will be donated to ACC research. It costs you nothing and keeps the research flowing. Find out more when you  click here . Enter "Adenoid Cystic Carcinoma Research Foundation" when asked to select a charitable organization.  
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