• Current Studies

    (Updated October 2020)

    ACCRF has compiled a list of clinical trials for ACC patients with advanced disease to consider. This website is updated periodically, but may not list all the pertinent and available trials. Patients may search on their own for recruiting ACC clinical trials on the ClinicalTrials.gov website.

    The clinical trials are broken down into four categories. The first three involve systemic therapy for patients with progressing recurrent/metastatic disease. The fourth involves localized therapy for patients with unresectable (inoperable) disease, typically the primary tumor or its recurrence.

    • Table 1 – Studies for all recurrent/metastatic ACC patients (usually phase II clinical trials)
    • Table 2 – Studies for recurrent/metastatic ACC patients with NOTCH-activated tumors as determined by tumor profiling (either phase I or II clinical trials)
    • Table 3 – Studies for recurrent/metastatic ACC patients incorporating tumor profiling prior to selecting a targeted drug (“basket studies”)
    • Table 4 – Studies for ACC patients with unresectable (inoperable), locally-advanced tumors

    In all cases, patients should consult with their physicians to discuss the appropriate course of action.

    Not all clinical trials have similarly strong scientific rationales for why the drugs should be effective in ACC. The most promising studies will involve drugs that (1) target the known mechanisms of action that drive ACC progression, (2) demonstrate activity in preclinical models of ACC, and (3) have reports of clinical benefit in an ACC patient from a case study or clinical trial. To assist patients and their physicians in appraising each clinical trial, we indicate whether the evidence supporting the scientific rationale is very strong, strong, solid or fair. In coming to an informed decision, each patient and consulting physician will have to consider additional factors, such as drug toxicity, cost, travel demands and biopsy requirements.

    The table below lists clinical trials that currently are recruiting ACC patients in particular or salivary gland cancer patients. Unless otherwise noted, these studies are for only ACC patients with recurrent or metastatic disease. Links are provided to descriptions of the drugs as well as the www.ClinicalTrials.gov summary of each study.

    Table 1 – Clinical Trials for All ACC Patients

    Compound Targets Sponsor Locations Scientific Rationale Info Link Contact
    Rivoceranib (Apatinib) VEGFR Elevar Therapeutics Multiple sites in Korea (Seoul & Goyang-si) and USA (Ann Arbor, Boston, Denver, Los Angeles, San Francisco & Tampa) Very Strong View Steven Norton, PhD
    1-801-303-7440 ext 275
    Axitinib and Avelumab VEGFR, PDGFR, KIT and PD-L1 Immunotherapy MD Anderson Houston, TX, USA Very Strong View Renata Ferrarotto, MD
    Lenvatinib and Pembrolizumab VEGFR and PD-1 Immunotherapy Memorial Sloan Kettering New York, NY, USA Very Strong View Alan Ho, MD, PhD
    GSK3326595 (for patients without prior systemic drugs) PRMT5 GSK Multiple sites in Canada, France, Netherlands and USA Strong View US: 877-379-3718 GSKClinicalSupportHD@gsk.com
    EU: +44(0)20 89904466 GSKClinicalSupportHD@gsk.com
    PRT-543 PRMT5 Prelude Therapeutics Houston, TX, USA Strong View Renata Ferrarotto, MD
    MYB DNA vaccine and Tislelizumab MYB and PD-1 immunotherapy Peter MacCallum Cancer Centre Melbourne, Australia Strong View Jayesh Desai
    +61 38559 7810
    Lutetium-177-PSMA Radioligand Therapy PSMA Radboud University Nijmegen, Netherlands Strong View Carla ML van Herpen, MD, PhD
    APG-115 with or without Carboplatin MDM2 University of Michigan Ann Arbor, MI, USA Solid View Paul L Swiecicki, MD 1-734-647-1017 pswiecic@med.umich.edu
    Ryan Drzewicki ryandrze@med.umich.edu
    Pembrolizumab and Docetaxel PD-1 Immunotherapy University of Chicago Chicago, IL, USA Solid View Alexander Pearson, MD, PhD
    Chidamide and Cisplatin HDAC Fudan University Shanghai, China Solid View Kai Xue, MD
    Chidamide HDAC Chinese Academy of Medical Sciences Beijing, China Fair View Mei Dong
    Nivolumab and Ipilimumab and Radiation PD-1
    and CTLA-4
    University of Washington Seattle, WA, USA Fair View Susan Masterson

    Approximately 25% of metastatic ACC patients have tumors with activating alterations in the NOTCH pathway (primarily in the NOTCH1 gene). These tumors behave more aggressively. Patients for whom tumor profiling has identified a NOTCH pathway alteration may wish to discuss the following studies with their physicians:

    Table 2 – Clinical Trials for ACC Patients with Activating NOTCH Alterations

    Compound Targets Sponsor Locations Scientific Rationale Info Link Contact
    AL101 NOTCH Ayala Pharmaceuticals Calgary, Alberta, Canada
    Hamilton, Ontario, Canada
    London, Ontario, Canada
    Villejuif, France
    Nijmegen, Netherlands
    Manchester, UK
    Aurora, CO, USA
    Baltimore, MD, USA
    Boston, MA, USA
    Houston, TX, USA
    Los Angeles, CA, USA
    Miami, FL, USA
    New York, NY, USA
    Tampa, FL, USA
    Seattle, WA, USA
    Strong View clinicaltrials@ayalapharma.com
    CB-103 NOTCH Cellestia Pharmaceuticals Amsterdam, Netherlands
    Maastricht, Netherlands
    Utrecht, Netherlands
    Barcelona, Spain
    Madrid, Spain
    Bellinzona, Switzerland
    Houston, TX, USA
    Strong View Pavel Pisa, MD

    Historically, clinical trials involved only one or two treatments. Some newer trials are incorporating tumor profiling to direct patients to many more potential treatments within the same study. These “basket trials” try to match targeted drugs to genomic alterations in each patient’s particular tumor. Some of these trials profile the patient’s tumor as party of the study (NCI-MATCH) while others will suggest treatment decisions based on existing tumor profiling reports (ASCO TAPUR). The clinical trials listed below are not specifically for ACC patients, but may be worth considering in consultation with a knowledgeable physician.

    Table 3 – “Basket” Clinical Trials Incorporating Tumor Profiling

    Compound Targets Sponsor Locations Scientific Rationale Info Link Contact
    NCI-MATCH Multiple targets National Cancer Institute Over 1,000 locations in USA Solid View View Info Link for details on each site
    ASCO TAPUR Multiple targets American Society of Clinical Oncology Over 20 locations in USA Solid View Pam Mangat, MS

    A small but significant subset of ACC patients have primary or recurrent tumors in the head and neck region that are unresectable (inoperable) due to their location near sensitive structures or previous treatments. In such cases, localized therapies such as radiation or drug injections may be appropriate.

    Table 4 – Studies for ACC Patients with Unresectable (Inoperable), Locally-Advanced Tumors

    Compound Modality Sponsor Locations Info Link Contact
    CV8102 (for superficial and readily-accessible head & neck tumors) Injection of TLR7/8 agonist CureVac Multiple sites in Germany View Thomas Eigentler, Prof. Dr.
    Apatinib and Proton Radiation (for inoperable or residual ACC) Radiation with systemic therapy Shanghai Proton and Heavy Ion Center Shanghai, China View Lin Kong, MD lin.kong@sphic.org.cn
    Jiyi Hu, MD jiyi.hu@sphic.org.cn
    Intensity-Modulated or Proton Radiation Therapy for Sinonasal Malignancy Radiation Massachusetts General Hospital Boston, MA, USA View Annie W Chan, MD
    Carbon Ion Only Irradiation vs Boost (ACCO) Radiation Heidelberg University Heidelberg, Germany View Klaus Herfarth, Prof. Dr.
    +49 6221 568201
    Randomized Carbon Ions vs Standard Radiotherapy for Radioresistant Tumors (ETOILE) Radiation Hospices Civils de Lyon Multiple sites in France View Pascal Pommier, MD
    (0)4 78 78 51 66 ext +33
    Download Selected Clinical Trials – Currently Open (Updated June 2020)