Adenoid Cystic Carcinoma Clinical Trials (April 2026): 17 Recruiting Interventional Studies
Last updated: April 25, 2026
Current Clinical Trial Landscape
Active research areas in 2026:
- NOTCH-targeted therapy — CB-103 (oral NOTCH inhibitor) in combination trials for NOTCH-mutant ACC
- Antibody-drug conjugates (ADCs) — targeting c-Kit, Nectin-4, B7-H4, TROP2, and EGFR on ACC cells
- Carbon ion radiotherapy — three recruiting trials for locally advanced/unresectable ACC
- Tyrosine kinase inhibitors — lenvatinib, axitinib, and novel multi-kinase inhibitors
- Immunotherapy combinations — PD-1/PD-L1 + VEGF bispecifics for salivary gland cancers
Standard of care: Surgery with or without adjuvant radiation for localized disease. For metastatic ACC, there are no FDA-approved targeted therapies — making clinical trials especially important. Chemotherapy (cisplatin, doxorubicin, cyclophosphamide) has limited efficacy. Lenvatinib is sometimes used off-label based on phase 2 data. Slow growth means some patients are monitored with active surveillance before starting systemic therapy.
Understanding Adenoid Cystic Carcinoma
ACC is a rare cancer (~1,200 new cases/year in the US) that most commonly arises in salivary glands (parotid, submandibular, minor salivary) but can also occur in the breast, skin, cervix, prostate, and trachea. It behaves differently from most cancers:
- Slow but relentless growth — ACC often grows slowly over years, but recurrence is common even 10-15 years after initial treatment
- Perineural invasion — ACC has a strong tendency to spread along nerves, which can cause pain and makes surgical margins challenging
- Late distant metastases — lung metastases are the most common site of spread, sometimes appearing many years after the primary tumor
- Histologic subtypes — solid pattern is most aggressive, cribriform is most common, tubular has the best prognosis. Subtype affects trial eligibility for some studies.
Key Biomarkers for Trial Eligibility
Molecular profiling is increasingly important for ACC — several biomarkers determine eligibility for targeted trials:
- NOTCH1/2 mutations — present in ~15-20% of ACC, associated with solid subtype and more aggressive behavior. Required for CB-103 trial (NCT05774899). NOTCH-mutant ACC may also respond to gamma-secretase inhibitors.
- MYB-NFIB fusion — the hallmark genetic event in ACC (~60-80%), driving the transcriptional program of the tumor. Not yet directly targetable but a diagnostic marker and potential future therapeutic target.
- c-Kit (CD117) expression — overexpressed in ~80-90% of ACC. Target for the ADC NN3201 (NCT06805825). Imatinib showed limited single-agent activity historically.
- EGFR expression — present in a subset of ACC. Relevant for EGFR-targeting ADCs like MRG003/becotatug vedotin.
- TrkA expression — target for VMD-928 trial. NTRK fusions (rare) are druggable with larotrectinib or entrectinib.
Only 17 ACC trials on ClinicalTrials.gov — we search 70+ by including salivary gland and solid tumor trials too.
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Recruiting Trials by Treatment Setting
Locally Advanced / Unresectable — Radiation Approaches
ACC is relatively radioresistant to conventional photon radiation. Carbon ion (heavy particle) therapy delivers higher biological effectiveness and may improve local control:
- Carbon ion radiotherapy:
- NCT05733910 - Carbon ion with simultaneous integrated boost
- NCT04214366 - Carbon ion only irradiation
- NCT02838602 - Carbon ions vs standard radiotherapy (randomized)
- SBRT for oligometastatic disease:
- NCT04883671 - Stereotactic body RT for oligometastatic ACC
Metastatic / Recurrent — Systemic Therapy
The most active area of ACC research. No standard second-line therapy exists, making trials critical:
- NOTCH-targeted therapy:
- NCT05774899 - CB-103 (oral NOTCH inhibitor) + lenvatinib or abemaciclib for NOTCH-mutant ACC (Phase 1/2)
- Antibody-drug conjugates (ADCs):
- NCT06805825 - NN3201 (c-Kit ADC) — targets the most commonly expressed protein in ACC
- NCT06891560 - Enfortumab vedotin (Nectin-4 ADC)
- NCT05377996 - XMT-1660 (B7-H4 ADC)
- NCT07521670 - Sacituzumab tirumotecan (TROP2 ADC) for recurrent/metastatic ACC (Phase 2)
- NCT07522879 - Becotatug vedotin (MRG003, EGFR ADC) + epirubicin as neoadjuvant (Phase 2)
- Tyrosine kinase inhibitors:
- Novel targeted agents:
- Immunotherapy:
- NCT03556228 - VMD-928 ± pembrolizumab (TrkA-overexpressing tumors)
- NCT06805617 - Ivonescimab (PD-1/VEGF bispecific) for salivary gland cancers
Showing selected notable trials. View all 17 recruiting interventional trials on ClinicalTrials.gov. ACC patients may also be eligible for broader salivary gland cancer trials and head and neck cancer trials.
Frequently Asked Questions
How do I find adenoid cystic carcinoma clinical trials I'm eligible for?
Enter your ACC details into ClinTrialFinder — including tumor location, NOTCH mutation status, histologic subtype, and prior treatments. The AI matches you with trials in minutes. ClinTrialFinder also searches broader salivary gland and head-and-neck cancer trials that may include ACC patients. No login required.
What ACC trials are currently recruiting?
There are 17 recruiting interventional trials for adenoid cystic carcinoma including NOTCH-targeted therapy (CB-103), antibody-drug conjugates targeting c-Kit, Nectin-4, B7-H4, and TROP2, carbon ion radiotherapy, tyrosine kinase inhibitors, and immunotherapy combinations.
Why are there so few ACC clinical trials?
ACC is a rare cancer (~1,200 cases/year in the US), which makes large clinical trials difficult to conduct. However, ACC patients may qualify for broader trials — many "salivary gland cancer," "head and neck cancer," and "advanced solid tumor" trials include ACC. ClinTrialFinder's basket trial discovery automatically finds these broader trials that may be relevant.
Should I get molecular profiling for adenoid cystic carcinoma?
Yes — molecular profiling can identify actionable targets. NOTCH1/2 mutations (~15-20%) qualify for the CB-103 trial. c-Kit expression (~80-90%) is relevant for the NN3201 ADC trial. Rare NTRK fusions are druggable with FDA-approved therapies (larotrectinib, entrectinib). Comprehensive genomic profiling (e.g., Foundation Medicine, Tempus) is recommended.
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