ACCRF Summer Update

ACCRF Update - August 2025

Historically, clinical trials in ACC have been challenging, rarely leading to responses (at least 30% tumor shrinkage) in more than 15% of patients. And results have been even more challenging for patients with ACC-I tumors which typically have solid histology, NOTCH-mutations, and poor prognosis.

At this year’s American Society of Clinical Oncology (ASCO) Annual Meeting in Chicago, biotech firm Mersana Therapeutics presented preliminary phase 1 clinical trial data for their antibody drug conjugate (ADC) Emiltatug ledadotin (Emi-Le; XMT-1660), which targets the B7-H4 protein that is highly prevalent in ACC-I tumors. Early data from the trial showed that 55.6% of ACC-I patients treated with this B7-H4 ADC experienced tumor responses—the highest level of clinical response ever seen for any drug tested in ACC.

This incredibly promising initial data is built on a concerted effort led by ACCRF and our research and biotech partners. Our “bench to bedside” holistic approach aims to identify drug targets in ACC tumors, validate their therapeutic potential in ACC models, and shepherd new, more effective treatments through to clinical trials in patients, ensuring that a solid and rigorous scientific foundation is the basis for all treatment options we pursue.

You can read more about the development of this new ADC as well as some of the many exceptional ACCRF grantees and industry partners who made it possible in the full feature on our website.

Shalil Giannone Joins ACCRF Board of Directors

In July of 2025, the ACCRF Board of Directors unanimously elected Shalil Giannone as the newest member of the Board. An active and prominent member of the ACC patient community for 15 years, Shalil has been a consummate partner in the work of ACCRF and an ally and champion to her fellow patients. Since her initial diagnosis in 2010, she has been attending and facilitating patient gatherings and helping to build community. After her ACC diagnosis, Shalil underwent a partial maxillectomy followed by radiation treatment. She is actively participating in the ACCRF-sponsored SOLAR trial at the Dana-Farber Cancer Institute for oligometastatic ACC tumors in her lungs.

Shalil became a Certified Public Accountant after earning her bachelor’s degree at the University of Central Florida. She has worked in both public and private accounting roles for over 30 years. Shalil holds additional certifications in Estate Planning and an AICPA Not for Profit certification. She has her own practice focused on assisting small nonprofit organizations with their accounting needs. Shalil resides in Charlotte, NC.

ACCRF is proud to welcome Shalil to the Board of Directors. You can learn more about Shalil in her Patient Champions feature from 2024.

Save the Date: Fall Patient Webinar

Save the date for ACCRF’s next Patient Webinar Saturday, October 11 at 12pm ET discussing Antibody Drug Conjugates (ADCs) and current open clinical trials for novel ACC treatments.

An invitation with registration details will be sent out in the coming weeks.

If you have questions or suggestions for topics of future patient webinars, please contact us at info@accrf.org.

You can watch all previous webinars in our Patient Webinar Archive or on the ACCRF YouTube Channel.

Research Updates

We are proud to support many oncologists in their ongoing work towards new treatments and a cure for ACC. The ACCRF research network continues to facilitate cutting-edge research and clinical trials that advance our understanding of the disease. Below are the latest updates in clinical trials, research publications from ACCRF grantees, and ACCRF-funded research grants.

Clinical Trial Updates

ACCRF’s Open Studies webpage lists clinical trials that may be appropriate for ACC patients after consulting their physicians. Listed studies below reflect any changes made since the previous email update.

Studies that have recently opened or begun enrolling patients:

BG-C9074 alone and in combination with Tislelizumab: The phase 1 trial testing BeiGene's B7-H4 ADC (BG-C9074) alone and in combination with a PD-1 antibody (Tislelizumab) is open to ACC-I patients. The clinical trial is open at multiple sites across the USA, Australia and China.

NN3201: The phase 1 trial testing Novelty Nobility's c-KIT ADC, NN3201, is open to ACC patients. The trial is open at multiple sites across the USA.

Published Articles

Below are the latest in research publications from ACCRF grantees. You can find these and many others on our Published Articles webpage.

Assessing the selective impact of histone modifying drugs on adenoid cystic carcinoma cells and their stem cell counterparts: ACCRF grantee Dr. Rogerio Castilho screened a library of histone modification drugs to assess their impact on cancer stem cells and other tumor cells in ACC. He identified histone deacetylases, histone methyltransferases, EZH2, c‑RET, and EGFR as key epigenetic targets.

Association of quality of life with mortality in patients with adenoid cystic carcinoma using an internationally-validated QoL questionnaire (EQ-5D-5L): ACCRF grantees Drs. Robert Metcalf, Guy Betts, and Laura Spurgeon shared the results of a quality of life survey filled out by 161 ACC patients. They found that lower quality of life scores is associated with a higher risk of death; however, it's still unclear if poor quality of life actually causes worse outcomes. Quality of life data has become increasingly important to support the approval and reimbursement of anti-cancer drugs for patients.

The Small Molecule Inhibitor of the Wnt-β-Catenin Pathway, CWP232291, Inhibits Tumor Growth in Preclinical Models of Salivary Gland Adenoid Cystic Carcinoma: ACCRF grantees Drs. Patrick Ha and Bhumsuk Keam showed that high levels of beta-catenin in ACC tumors correlate with reduced survival. They also demonstrated that a drug targeting the Wnt-β-catenin signaling pathway was active in preclinical models of ACC, suggesting a potential new therapeutic approach.

TROP2 Expression in Salivary Gland Adenoid Cystic Carcinoma (ACC) According to Histologic Subtype: Therapeutic Implications: ACCRF grantees Drs. Ferrarotto, El-Naggar, Mitani, Hoff, and de Sousa studied 165 ACC tumors and found that 97% were positive for a protein called TROP2. This protein is the target of a drug called sacituzumab govitecan, which is a type of antibody-drug conjugate (a drug that delivers chemo directly to cancer cells). They also found that ACC-II tumors, which typically contain cribriform and tubular histology and do not have NOTCH mutations, had the highest levels of TROP2. These findings helped support the launch of a clinical trial testing sacituzumab govitecan in ACC patients (NCT05884320).

A macrophage-predominant immunosuppressive microenvironment and therapeutic vulnerabilities in advanced salivary gland cancer: ACCRF grantees Dr. Damien Rieke and Dr. Luc Morris published a study on the immune environment in advanced salivary gland cancers. Their research confirms that ACC tumors have very little immune activity (called "immunologically cold") and shows that high levels of the B7-H4 protein (also known as VTCN1) is found in both ACC-I and ACC-II tumors. Antibody drug conjugates designed to deliver chemotherapy to cells expressing high levels of B7-H4 are currently being tested in clinical trials.

ACCRF is excited to announce the launch of our Instagram @TheACCRF! Follow us here for patient resources, event updates, and the latest in research and education!

ACC Physicians List

ACCRF is pleased to provide the ACC Physicians List, containing up-to-date information for over 850 physicians globally with experience treating ACC.

This fully searchable database allows patients to locate physicians by name, location, institution, or field of expertise to help inform their decisions in selecting doctors. Features include a link to each doctor’s institutional website (where available) and a link to the National Physicians Index (NPI) listing for each doctor based in the USA which provides current information on practice locations and status.

This list is constantly evolving, and we rely on patient input to ensure that our database is as comprehensive and current as possible.

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 on the list has retired
Your doctor on the list has moved away from where s/he is listed

Your guidance will ease the path for future ACC patients. We are particularly interested in countries and regions with relatively few available physicians listed.

We encourage patients with advanced, metastatic, or complex cases of ACC to visit our Second Opinions Resource for more information on specialists.

ACCRF’s work is only possible through the generous support of community members like you. With increased uncertainty in the research funding landscape, our efforts to discover new, better treatments for ACC are more critical than ever. Please give today and help us continue to advance groundbreaking research that directly impacts the lives of patients.

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