Adenoid Cystic Carcinoma - Market Insight, Epidemiology, and Market Forecast - 2034

Key Highlights:

• Adenoid cystic carcinoma (ACC) is a rare type of cancer that originates primarily in glandular tissues, most commonly in the salivary glands of the head and neck, but it can also occur in other secretory glands throughout the body, including the breast, lacrimal gland, lung, trachea, paranasal sinuses, and more.
• Adenoid cystic carcinoma is considered the third most common malignant salivary gland tumor overall, and is especially predominant in the submandibular gland.
• Adenoid cystic carcinoma is often diagnosed in one of the three major salivary glands parotid, submandibular, sublingual gland, but more than half of ACC cases arise from the minor salivary glands which surround the hard/soft palate, inside the cheeks, nose, sinus, tongue, larynx, pharynx and more.
• The United States accounts for the largest market size of adenoid cystic carcinoma, in comparison to EU4 (Germany, Spain, Italy, France), the United Kingdom, and Japan.
• Currently, there are no approved systemic therapies tailored specifically for adenoid cystic carcinoma, highlighting a significant unmet need and underscoring the urgency for innovative treatment options in this rare and resilient cancer.
• Companies like Elevar Therapeutics, Remix Therapeutics, Rgenta Therapeutics, Ascentage Pharma, Mersana Therapeutics, OncoC4, and VM Oncology are driving a new wave of innovation in adenoid cystic carcinoma, using cutting-edge platforms from RNA-targeting to antibody-drug conjugates to tackle this rare cancer.
• The pipeline for adenoid cystic carcinoma is steadily expanding, with promising candidates such as alrizomadlin, Gotistobart (ONC-392), REM-422, RGT-61159, and others currently mostly in early or mid stages of clinical development.
• In October 2024, Rgenta Therapeutics announced the dosing of the first patients in a Phase I trial of RGT-61159, an oral small molecule targeting RNA regulation. The drug is being developed for adenoid cystic carcinoma, colorectal cancer, acute myeloid leukemia, and other solid tumors.
• At the AACR Annual Meeting 2025, Remix Therapeutics presented new preclinical data in Session PO.ET09.10 highlighting REM-422, showing potent anti-tumor activity in xenograft models of adenoid cystic carcinoma underscoring its promise as a novel therapeutic strategy.
• In June 2025, Ascentage Pharma announced new Phase II clinical data for its MDM2-p53 inhibitor, alrizomadlin (APG-115), at the 61st ASCO Annual Meeting. The study evaluated alrizomadlin alone or with PD-1 inhibitor toripalimab in patients with advanced adenoid cystic carcinoma and other solid tumors.

DelveInsight's "Adenoid cystic carcinoma (ACC) - Market Insights, Epidemiology and Market Forecast - 2034" report delivers an in-depth understanding of ACC, historical and forecasted epidemiology as well as the ACC market trends in the United States, EU4 (Germany, Spain, Italy, and France) and the United Kingdom, and Japan.

Adenoid cystic carcinoma (ACC) market report provides real-world prescription pattern analysis, emerging drugs, market share of individual therapies, and historical and forecasted 7MM ACC market size from 2020 to 2034. The report also covers current ACC treatment practices/algorithms and unmet medical needs to curate the best opportunities and assess the market's underlying potential.

Geography Covered:

• The United States
• EU4 (Germany, France, Italy, and Spain) and the United Kingdom
• Japan

Adenoid Cystic Carcinoma (ACC) Understanding and Treatment Algorithm

Adenoid Cystic Carcinoma (ACC) Overview, Country-Specific Treatment Guidelines, and Diagnosis

Adenoid cystic carcinoma is a rare cancer that most commonly arises in the glandular tissues of the head and neck, particularly the salivary glands, but it can also develop in other areas such as the skin, breast, and respiratory tract. ACC tends to grow slowly but is unpredictable, with a potential to recur many years after initial treatment and to spread (metastasize), most often to the lungs, liver, or bones. It is more frequently diagnosed in people aged 40 to 60 and is slightly more common in females. The exact cause of ACC is unknown, but it is believed to result from genetic changes acquired during a person's lifetime rather than inherited mutations.

Diagnosis of ACC begins with a thorough medical history and physical examination, focusing on the location and characteristics of any lumps or symptoms. Imaging studies such as CT, MRI, PET, and ultrasound scans are used to determine the size, location, and extent of the tumor, as well as to check for spread to other parts of the body. A definitive diagnosis is made by biopsy, where a tissue sample is examined under a microscope to identify the characteristic features of ACC. Biopsies can be performed with fine needle aspiration, core needle, or surgical methods, depending on the tumor's location and accessibility. Overactivation of the MYB oncogene has been described as a hallmark of ACC and is noted in over 90% of ACC cases.

Adenoid Cystic Carcinoma (ACC) Treatment

The primary treatment for ACC is surgical removal of the tumor, often followed by radiation therapy to reduce the risk of recurrence. Surgery aims to excise the tumor with clear margins, but due to the tumor's tendency to invade nerves and surrounding tissues, complete removal can be challenging. Radiation therapy may be used as an adjunct or, in cases where surgery is not feasible, as the main treatment. Chemotherapy is generally less effective for ACC, as these tumors are often resistant to standard chemotherapeutic agents. However, targeted therapies and participation in clinical trials may be considered for advanced or recurrent cases, and management typically involves a multidisciplinary team approach tailored to the individual patient.

Adenoid Cystic Carcinoma (ACC) Epidemiology

The ACC epidemiology chapter in the report provides historical as well as forecasted data in the 7MM covering the United States, EU4 countries (Germany, France, Italy, and Spain), the United Kingdom, and Japan from 2025 to 2034. The ACC epidemiology is segmented with detailed insights into Incident Cases, Age-specific Cases, Gender-specific Cases, Site-specific Cases, Treated Cases of ACC.

• As per the Adenoid cystic carcinoma Research Foundation, It is estimated that approximately 11,000 people are living with ACC in the US.
• Although ACC can occur at any age, it most frequently presents in middle-aged to older adults, with an average age at diagnosis around 52 years.
• Approximately 80% of ACC patients are diagnosed with a primary tumor in the head or neck area. ACC of the salivary glands, the most common form of the disease, is thought to account for ~25% of all salivary gland malignancies.
• Survival rates are relatively high at five years (~80%), but decline significantly over longer periods due to late recurrences and metastases.

Adenoid Cystic Carcinoma (ACC) Drug Chapters

The section dedicated to drugs in the ACC report provides an in-depth evaluation of pipeline drugs (Phase III and Phase II) related to ACC. The drug chapters section provides valuable information on various aspects related to clinical trials of ACC, such as the pharmacological mechanisms of the drugs involved, designations, approval status, patent information, and a comprehensive analysis of the pros and cons associated with each drug. Furthermore, it presents the most recent news updates and press releases on drugs targeting ACC.

Emerging Therapies

Gotistobart (ONC-392): OncoC4

ONC-392 is the first acid pH-sensitive anti-CTLA-4 antibody, designed to bind CTLA-4 at physiological and tumor pH but release it in acidic environments (pH <6.0). This preserves CTLA-4's normal recycling in healthy tissues, reducing toxicity, while enabling effective depletion of tumor-infiltrating Tregs. Its unique mechanism enhances safety and efficacy, offering a superior therapeutic index over traditional CTLA-4 therapies.

ONC-392 is currently being evaluated in a Phase II cohort of the PRESERVE-001 trial (NCT04140526) specifically for adenoid cystic carcinoma, aiming to assess its safety and efficacy as a novel, next-generation anti-CTLA-4 immunotherapy in this hard-to-treat cancer.

Alrizomadlin (APG-115): Ascentage Pharma

Alrizomadlin (APG-115) is an oral, selective small molecule inhibitor of MDM2, designed to disrupt the MDM2-p53 interaction. By inhibiting MDM2, alrizomadlin stabilizes and activates the tumor suppressor protein p53, promoting apoptosis in cancer cells.

At ASCO 2025, Ascentage Pharma presented new Phase II clinical data of alrizomadlin in patients with ACC that demonstrated an objective response rate (ORR) of 16.7% and a disease control rate (DCR) of 100%. These results suggest that the targeted inhibition of the MDM2-p53 pathway has antitumor activity in ACC; therefore, it can potentially offer a new treatment strategy to patients with ACC.

Adenoid Cystic Carcinoma (ACC) Market Outlook

The ACC market is expected to grow steadily through the next decade, propelled by innovation in diagnostics and therapeutics, increased clinical trial activity, and a greater focus on personalized medicine. However, challenges such as high treatment costs and the rarity of the disease will continue to shape the market landscape.

• Significant unmet needs remain in advanced or metastatic disease, highlighting the need for innovation, as no FDA, EMA, or PMDA approved treatments exist.
• Surgery remains the primary treatment, especially for salivary gland ACC, often followed by radiation therapy if there is a risk of recurrence or incomplete removal.
• During the forecast period, pipeline candidates such as Alrizomadlin, Gotistobart, REM-422, RGT-61159, and others are expected to drive the rise in ACC market size.

In a nutshell, many potential therapies are being investigated to manage ACC. Even though it is too soon to comment on the above-mentioned promising candidate to enter the market during the forecast period (2025-2034), it is safe to assume that the future of this market is bright. Eventually, these drugs will create a significant difference in the landscape of ACC in the coming years. The treatment space is expected to experience a significant positive shift in the coming years owing to the improvement in healthcare spending worldwide.

KOL Views

To keep up with the real-world scenario in current and emerging market trends, we take opinions from Key Industry leaders working in the domain through primary research to fill the data gaps and validate our secondary research. Industry Experts were contacted for insights on the evolving treatment landscape, patient reliance on conventional therapies, patient therapy switching acceptability, and drug uptake, along with challenges related to accessibility.

DelveInsight's analysts connected with 25+ KOLs to gather insights; however, interviews were conducted with 15+ KOLs in the 7MM. Centers such as Nationwide Children's Hospital, University of Munich, the University of Tokyo, University of Brescia, National Cancer Institute, and University of Minnesota, University Hospitals Dorset, NHS Foundation Trust, North Shore University Health System, Evanston, Illinois, etc., were contacted. Their opinion helps understand and validate current and emerging treatment patterns of ACC. This will support the clients in potential upcoming novel treatments by identifying the overall scenario of the market and the unmet needs.

Qualitative Analysis

We perform Qualitative and market Intelligence analysis using various approaches, such as SWOT analysis and Conjoint Analysis. In the SWOT analysis, strengths, weaknesses, opportunities, and threats in terms of gaps in disease diagnosis, patient awareness, physician acceptability, competitive landscape, cost-effectiveness, and geographical accessibility of therapies are provided.

Conjoint Analysis analyzes multiple approved and emerging therapies based on relevant attributes such as safety, efficacy, frequency of administration, route of administration, and order of entry. Scoring is given based on these parameters to analyze the effectiveness of therapy.

In efficacy, the trial's primary and secondary outcome measures are evaluated; for instance, in event-free survival, one of the most important primary outcome measures is objective response rate (ORR), disease control rate (DCR), Maximum Tolerated Dose (MTD), and Treatment Emergent Adverse Events (TEAEs).

Further, the therapies' safety is evaluated, wherein the acceptability, tolerability, and adverse events are majorly observed, and it sets a clear understanding of the side effects posed by the drug in the trials. In addition, the scoring is also based on the probability of success and the addressable patient pool for each therapy. According to these parameters, the final weightage score and the ranking of the emerging therapies are decided.

Scope of the Report:

• The report covers a segment of key events, an executive summary, a descriptive overview of ACC, explaining its causes, signs and symptoms, pathogenesis, and currently available therapies.
• Comprehensive insight has been provided into the epidemiology segments and forecasts, the future growth potential of diagnosis rate, and disease progression, along with country-specific treatment guidelines.
• Additionally, an all-inclusive account of current therapies.
• A detailed review of the ACC market, historical and forecasted market size, market share by therapies, detailed assumptions, and rationale behind our approach is included in the report, covering the 7MM drug outreach.
• The report provides an edge while developing business strategies, by understanding trends, through SWOT analysis and expert insights/KOL views, patient journey, and treatment preferences that help in shaping and driving the 7MM ACC market.

Adenoid Cystic Carcinoma (ACC) Report Insights

• Patient Population
• Therapeutic Approaches
• ACC Market Size and Trends
• Existing and future Market Opportunity

Adenoid Cystic Carcinoma (ACC) Report Key Strengths

• Ten Years Forecast
• 7MM Coverage
• ACC Epidemiology Segmentation
• Inclusion of country-specific treatment guidelines
• KOL's feedback on approved and emerging therapies
• Key Cross Competition
• Drugs Uptake and Key Market Forecast Assumptions

Adenoid Cystic Carcinoma (ACC) Report Assessment

• Current Treatment Practices
• Unmet Needs
• Pipeline Product Profiles
• Market Attractiveness
• Qualitative Analysis (SWOT and Conjoint Analysis)

FAQs:

• What is the growth rate of the 7MM ACC treatment market?
• What was the ACC total market size, the market size by therapies, market share (%) distribution in 2020, and what would it look like in 2034? What are the contributing factors/key catalysts for this growth?
• Is there any unexplored patient setting that can open the window for growth in the future?
• What are the pricing variations among different geographies for approved and off-label therapies?
• How would the market drivers, barriers, and future opportunities affect the market dynamics and subsequent analysis of the associated trends? Although multiple expert guidelines recommend testing for targetable mutations before therapy initiation, why do barriers to testing remain high?
• What are the current options for the treatment of ACC?
• What are the recent novel therapies, targets, mechanisms of action, and technologies developed to overcome the limitations of existing therapies?
• Patient/physician acceptability in terms of preferred treatment options as per real-world scenarios?
• What are the country-specific accessibility issues of expensive, recently approved therapies?

Reasons to buy:

• The report will help in developing business strategies by understanding the latest trends and changing treatment dynamics driving the ACC Market.
• Insights on patient burden/disease prevalence, evolution in diagnosis, and factors contributing to the change in the epidemiology of the disease during the forecast years.
• Understand the existing market opportunities in varying geographies and the growth potential over the coming years.
• Distribution of historical and current patient share based on real-world prescription data along with reported sales of approved products in the US, EU4 (Germany, France, Italy, and Spain), the United Kingdom, and Japan.
• Highlights of access and reimbursement policies of approved therapies, barriers to accessibility of expensive off-label therapies, and patient assistance programs.
• To understand Key Opinion Leaders' perspectives around the accessibility, acceptability, and compliance-related challenges of existing treatment to overcome barriers in the future.
• Detailed insights on the unmet needs of the existing market so that the upcoming players can strengthen their development and launch strategy.