ACCRF Research Update
May 2022
ACCRF Board of Directors Welcomes Dr. Beth Moore Wehlage
The ACCRF Board of Directors consists of ACC patients or their family members who provide strategic guidance to the foundation's staff. Directors volunteer their legal, accounting, marketing, medical, financial, managerial, communications and public affairs expertise to help the foundation pursue its mission. Along with the Scientific Advisory Board, the Board of Directors is an essential component of the foundation's leadership.

ACCRF is very pleased to welcome Elizbeth (Beth) Moore Wehlage, MD, to the Board of Directors. Beth is a retired obstetrician-gynecologist and an active member of various ACC patient support groups, including serving as one of the moderators for the “ACC Warriors” Facebook group. Her compassionate and educated feedback has helped bring comfort to many ACC patients over the years. Beth joins the ACCRF Board of Directors with a special interest in patient-centered communications.
ACCRF Patient Webinar #2: Systemic Therapy in ACC
Last fall, ACCRF initiated a series of webinars to educate the ACC patient community about ACC and its treatment options. The first patient webinar on November 6, 2021, provided an overview of the status of ACC research.

The second patient webinar took place on May 7, 2022. The topic was "Systemic Therapy in ACC" and featured two medical oncologists with extensive ACC experience. Drs. Alan Ho and Glenn Hanna provided answers to questions from patients about advanced ACC that requires systemic therapy (drugs).

Both discussions are now available on ACCRF's new Patient Webinar Archive. Be sure to sign up for email updates to learn about upcoming webinars.
Clinical Trial Updates
Clinical trial activity remains high for ACC patients dealing with recurrent or metastatic disease that is progressing. ACCRF provides a list of clinical trials on the Open Studies webpage that may be appropriate for ACC patients to consider in consultation with their oncologists. In addition, the Completed Studies webpage reviews the history of systemic therapy (drugs) in ACC. Here we review some of the more recent developments in clinical trials.

Studies that recently opened or restarted recruitment:

  • P-PSMA-101 CAR T Cell Therapy – Building on the finding that a large proportion of ACC tumors express the cell surface protein PSMA, this study assesses the safety and activity of the introduction of immune cells engineered to recognize PSMA-positive cells. The clinical trial recently opened at various sites in the USA, including Boston, MA, Denver, CO, Duarte, CA, San Diego, CA and San Francisco, CA. You may read more about how ACCRF helped shepherd this concept to the clinic in the next section below.
  • Stereotactic Body Radiation Therapy (SBRT) trial for oligometastatic disease - Originally designed for patients with 3 or fewer metastases, this study recently expanded its inclusion criteria to accept ACC patients with up to 5 metastases. The study is open in Boston, MA, USA. Eventually, sites may open in Chicago, IL, and San Francisco, CA, USA.
  • AL101 - The original phase II clinical trial of AL101 for NOTCH-activated ACC patients has ended recruitment and will report its findings next month. However, a new "window-of-opportunity" study will test NOTCH inhibitor AL101 treatment before surgery in NOTCH-activated ACC patients. The trial recently opened in Houston, TX, USA.
  • APG-115 – This study is testing an MDM2 inhibitor in ACC patients that have tumors without p-53 alterations. The clinical trial's two arms closed last year to assess the preliminary data and then this year re-opened one arm with APG-115 alone (the carboplatin combination arm was discontinued). The study is in Ann Arbor, MI, USA.
  • Elraglusib - This study is testing a GSK3b inhibitor (in combination with a chemotherapy, Carboplatin) in salivary gland cancer patients. It recently opened in Boston, MA, USA.
  • Pembrolizumab and Pemetrexed - This study is testing an immune checkpoint inhibitor in combination with a chemotherapy. It recently opened in Rochester, MN, and Scottsdale, AZ, USA.

Studies that completed their enrollment:

  • GSK3326595 - Following a 21% partial response rate observed in ACC patients during the initial phase 1 setting, this expansion cohort testing a PRMT5 inhibitor recently completed enrollment.
  • PRT543 - The phase 1b clinical study testing this PRMT5 inhibitor completed enrollment at several sites in the USA.
  • Axitinib and Avelumab - The phase 2 study testing the combination of a multi-tyrosine kinase inhibitor and a PD-L1 inhibitor recently completed enrollment in Houston, TX. The preliminary findings will be reported next month.
  • Rivoceranib (Apatinib) - The phase 2 study off this multi-tyrosine kinase inhibitor recently completed enrolment in many sites across the USA and Korea. The preliminary findings will be reported next month.

We anticipate that the upcoming Annual Meetings of the American Society of Clinical Oncology (ASCO) in June will include updates on many clinical trials, including some mentioned above. ACCRF will share the findings from those reports in future email updates.
ACCRF's Goal: Shepherding Ideas from the Lab to ACC Clinical Trials
ACCRF’s mission is to accelerate the development of better treatments for ACC patients. We do that by investing in early basic research all the way through to preclinical drug screening and clinical trials. The foundation acts as a shepherd, coaxing and corralling scientists, drug companies and medical oncologists for the benefit of ACC patients. There are many examples but we will highlight the most recent one.

Six years ago, ACCRF recognized the importance of the finding that salivary glands and ACC tumors sometimes express prostate specific membrane antigen (PSMA) cell surface proteins. We funded two investigators to formally assess PSMA levels in ACC tumors, starting with Dr. Ana Ponce Kiess (Johns Hopkins University) in 2016 and then Carla van Herpen (Radboud University) in 2017. By 2020, Dr. van Herpen's work demonstrated that PSMA tracer uptake occurs in 93% of ACC patients and may help to detect smaller metastatic lesions that are missed by traditional CT scans. ACCRF then co-funded (with the Dutch Cancer Institute) a clinical trial of PSMA radioligand therapy in ACC patients at Radboud University. Subsequently, a similar study opened in Beijing, China.

Validating PSMA as a therapeutic target in ACC also creates a strong rationale to apply other types of PSMA-targeted therapies to ACC patients, even if they were originally intended for prostate cancer patients. One example is the P-PSMA-101 CAR T Cell Therapy trial that recently opened in salivary gland cancer patients. ACCRF approached various biopharmaceutical companies, explaining the relevance of PSMA to ACC, including Poseida Therapeutics that is developing the PSMA CAR T cell immunotherapy. Without the strong advocacy of ACCRF, ACC patients likely would have been bypassed.

As more PSMA-targeted therapies enter clinical testing in the future, ACCRF will continue to leverage basic science and access to our preclinical models and physician network to help bring new, promising therapies to ACC patients. To learn more about PSMA and other drug targets in ACC, visit our Molecular Targets webpage.
ACC Physicians List
Choosing the right doctor is a crucial decision. However, finding doctors experienced in treating ACC can be challenging. In order to help ACC patients make an informed decision in selecting doctors, the Adenoid Cystic Carcinoma Research Foundation (ACCRF) and the Adenoid Cystic Carcinoma Organization International (ACCOI) collaborate to offer an ACC Physicians List of doctors who have treated ACC patients.
 
We are always looking to improve the list! Please email us at info@accrf.org if any of the following apply:

  • You feel your doctor is knowledgeable about ACC and should be added to the list
  • Your doctor has retired
  • Your doctor has moved away from where s/he is listed
 
Your guidance will ease the path for future ACC patients. We are particularly interested in cities, regions and countries with relatively few available physicians listed.
 
Donate Every Time You Make a Purchase on Amazon
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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