Adrenocortical Carcinoma (ACC) - 7MM Market Insights, Epidemiology, And Forecast - 2036 Novel Therapies And Earlier Diagnosis Fuel 15% CAGR Through 2036
Dublin, Sept. 30, 2026 (GLOBE NEWSWIRE) -- "Adrenocortical Carcinoma - Market Insight, Epidemiology, and Market Forecast - 2036" has been added to ResearchAndMarkets.com's offering.
The Adrenocortical Carcinoma (ACC) market across the seven major markets (7MM) - the United States, Germany, France, Italy, Spain, the United Kingdom, and Japan - was valued at approximately USD 23 million in 2025. The market is projected to expand at a CAGR of approximately 15% from 2026-2036, supported by advances in diagnosis, increasing disease awareness, continued research activity, and the development of targeted therapies and immunotherapies. The United States represented the largest regional market, generating approximately USD 10 million in 2025.
ACC is a rare, aggressive malignancy associated with poor prognosis, high recurrence rates, and limited systemic treatment options. Genetic mutations and hereditary syndromes may contribute to disease development. The oncocytic variant accounts for approximately 10% of adrenal cortical tumors, while myxoid and sarcomatoid variants remain exceedingly rare. Under the 2022 WHO classification, oncocytic ACC is characterized by oncocytic tumor cells comprising more than 90% of the tumor mass.
ACC Epidemiology and Patient Burden
Epidemiological analysis indicates that the United States accounted for approximately 30% of incident ACC cases in the 7MM in 2025. Around 60% of cases occur in females, demonstrating a notable gender disparity. Incidence increases with age, with the largest proportion reported among individuals aged over 60 years, followed by those aged 40-60 years.
Stage IV disease represents approximately 35% of ACC cases in the 7MM, while Stage I accounts for about 10%. The high proportion of advanced-stage diagnoses reflects aggressive disease progression, delayed identification, and the absence of dependable screening methods. Earlier detection, validated diagnostic markers, and prognostic biomarkers remain critical unmet needs.
ACC Diagnosis and Treatment Landscape
ACC diagnosis incorporates hormonal evaluation, contrast-enhanced CT, MRI, and, when appropriate, PET imaging to assess tumor function, size, local invasion, and metastatic spread. Histopathological analysis following surgery or biopsy provides definitive confirmation. The ENSAT staging system is widely used to classify disease from Stage I through Stage IV and support treatment planning.
Surgical resection remains the primary potentially curative treatment for localized ACC. For advanced, metastatic, recurrent, or inoperable disease, systemic therapy is generally required. Mitotane (LYSODREN), distributed by Esteve Pharmaceuticals, is the only US FDA-approved drug specifically indicated for ACC. It is used as adjuvant therapy after surgery and in advanced disease, either alone or with cytotoxic chemotherapy.
The EDP-M regimen - etoposide, doxorubicin, cisplatin, and mitotane - remains a principal systemic treatment for advanced ACC. Radiation therapy may also support local disease control or recurrence management. Despite these approaches, treatment outcomes remain limited, reinforcing demand for therapies that improve survival, control hormone excess, reduce toxicity, and preserve quality of life.
ACC Pipeline and Competitive Landscape
The ACC pipeline includes early-stage programs from Cytovation, Orphagen Pharmaceuticals, Daiichi Sankyo, and other organizations. Investigational therapies are pursuing immunomodulatory, molecularly targeted, and endocrine pathway approaches.
- CY-101/Getacatetide: Cytovation is developing getacatetide as a membranolytic inhibitor of the Wnt/?-catenin pathway. Early antitumor activity was reported in the Phase I CICILIA trial, particularly in tumors with dysregulated Wnt/?-catenin signaling. CY-101 has received Orphan Drug Designation in the United States for ACC, while UK authorities approved the clinical trial application for a Phase II study. OR-449: Orphagen Pharmaceuticals is developing OR-449 as an orally-bioavailable antagonist of steroidogenic factor-1 (SF-1, NR5A1) for adult and pediatric ACC and other tumors with elevated SF-1 expression. The US FDA granted OR-449 Rare Pediatric Disease Designation for pediatric ACC. DS-9051b/DS-9051: This investigational program may follow a biomarker-driven development and adoption pathway, potentially focusing on molecularly defined patient populations.
In July 2025, Cytovation reported that the WHO International Nonproprietary Names Expert Committee approved getacatetide as the International Nonproprietary Name for CY-101. In April 2025, the company raised USD 6 million to advance a multinational Phase II ACC trial, with initial clinical readouts expected in 2026. Cytovation also entered a collaboration with Cancer Research UK and the Norwegian Cancer Society in January 2025 to support the program.
ACC Market Growth Drivers and Unmet Needs
Improved imaging, hormonal testing, and clinical awareness are increasing diagnostic accuracy and expanding the identifiable treatment population. At the same time, substantial unmet need continues to stimulate research and investment. Priority gaps include limited treatment choices, high recurrence, advanced-stage diagnosis, insufficient diagnostic and prognostic markers, small clinical trial populations, treatment-related toxicity, hormonal complications, and psychosocial burden.
Drug uptake during 2026-2036 is expected to be gradual because of the limited patient population and the early development status of many pipeline candidates. Mitotane is likely to remain the treatment backbone, while successful emerging therapies could gain adoption based on clinical efficacy, safety, biomarker selection, administration requirements, pricing, reimbursement, and positioning within treatment guidelines.
Report Coverage and Strategic Value
The Adrenocortical Carcinoma (ACC) - Market Insights, Epidemiology and Market Forecast - 2036 report provides historical and forecasted analysis for 2022-2036 across the 7MM. Coverage includes ACC epidemiology, patient burden, treatment algorithms, marketed and emerging therapies, market size, therapy-specific market share, drug uptake, peak patient share, pricing, reimbursement, competitive positioning, and commercial opportunities.
The analysis incorporates bottom-up epidemiology-based forecasting, SWOT analysis, conjoint analysis, secondary research, and primary insights from more than 15 key opinion leaders and subject matter experts. Clinical attributes such as efficacy, safety, tolerability, route and frequency of administration, order of entry, probability of success, and addressable patient population are assessed to compare emerging therapies.
- 11-year ACC market forecast across the United States, Europe, and Japan Country-level epidemiology and patient population projections ACC pipeline analysis and emerging drug profiles Treatment addressable market and therapy uptake forecasts Pricing, analogue assessment, reimbursement, and market access analysis Competitive landscape, unmet needs, and strategic growth opportunities Expert perspectives on clinical practice, accessibility, adherence, and therapy adoption
The ACC market outlook remains shaped by limited approved treatment options and a developing pipeline. However, rising awareness, improved diagnostic capabilities, biomarker-driven research, and investment in targeted and immuno-oncology strategies are expected to create meaningful clinical and commercial opportunities through 2036.
Key Topics Covered:
1. Key Insights
2. Report Introduction
3. Executive Summary
4. Key Events
4.1. Upcoming Key Catalyst
4.2. Key Conferences And Meetings
4.3. Key Transactions And Collaborations
4.4. News Flow
5. Epidemiology and Market Methodology of Adrenocortical Carcinoma (ACC)
6. Adrenocortical Carcinoma (ACC) Market Overview at a Glance
6.1. Clinical Landscape Analysis (By Molecule Type, Phase, and Route of Administration [ROA])
6.2. Market Share of Adrenocortical Carcinoma (ACC) By Therapies (%) in the 7MM in 2025
6.3. Market Share of Adrenocortical Carcinoma (ACC) By Therapies (%) in the 7MM in 2036
7. Disease Background And Overview of Adrenocortical Carcinoma (ACC)
7.1. Introduction
7.2. Causes
7.3. Signs And Symptoms
7.4. Diagnosis
7.4.1. Differential Diagnosis
7.4.2. Diagnostic Algorithm
7.4.3. Diagnostic Guidelines
7.5. Treatment and Management
7.5.1. Treatment Algorithm
7.5.2. Treatment Guidelines
8. Epidemiology and Patient Population of Adrenocortical Carcinoma (ACC)
8.1. Key Findings
8.2. Assumption and Rationale
8.3. Total Incident Cases of Adrenocortical Carcinoma (ACC) in the 7MM
8.4. The United States
8.4.1. Total Incident Cases of Adrenocortical Carcinoma (ACC) in the United States
8.4.2. Stage-specific Incident Cases of Adrenocortical Carcinoma (ACC) in the United States
8.4.3. Gender-specific Incident Cases of Adrenocortical Carcinoma (ACC) in the United States
8.4.4. Age-specific Incident Cases of Adrenocortical Carcinoma (ACC) in the United States
8.5. EU4 and the UK
8.5.1. Total Incident Cases of Adrenocortical Carcinoma (ACC) in EU4 and the UK
8.5.2. Stage-specific Incident Cases of Adrenocortical Carcinoma (ACC) in EU4 and the UK
8.5.3. Gender-specific Incident Cases of Adrenocortical Carcinoma (ACC) in EU4 and the UK
8.5.4. Age-specific Incident Cases of Adrenocortical Carcinoma (ACC) in EU4 and the UK
8.6. Japan
8.6.1. Total Incident Cases of Adrenocortical Carcinoma (ACC) in Japan
8.6.2. Stage-specific Incident Cases of Adrenocortical Carcinoma (ACC) in Japan
8.6.3. Gender-specific Incident Cases of Adrenocortical Carcinoma (ACC) in Japan
8.6.4. Age-specific Incident Cases of Adrenocortical Carcinoma (ACC) in Japan
9. Patient Journey of Adrenocortical Carcinoma (ACC)
10. Marketed Therapies
10.1. Marketed Competitive Landscape of Adrenocortical Carcinoma (ACC)
10.2. Mitotane (LYSODREN): Esteve
10.2.1. Product Description
10.2.2. Regulatory Milestones
10.2.3. Other Developmental Activities
10.2.4. Summary of Pivotal Trials
10.2.5. Analyst Views
11. Emerging Therapies
11.1. Emerging Competitive Landscape of Adrenocortical Carcinoma (ACC)
11.2. CY-101/Getacatetide: Cytovation
11.2.1. Product Description
11.2.2. Other Developmental Activities
11.2.3. Clinical Development
11.2.3.1. Clinical Trial Information
11.2.4. Safety and Efficacy
11.2.5. Analyst Views
11.3. DS9051b: Daiichi Sankyo
11.3.1. Product Description
11.3.2. Other Developmental Activities
11.3.3. Clinical Development
11.3.3.1. Clinical Trial Information
11.3.4. Safety and Efficacy
11.3.5. Analyst Views
12. Adrenocortical Carcinoma (ACC): Seven Major Market Analysis
12.1. Key Findings
12.2. Market Outlook of Adrenocortical Carcinoma (ACC)
12.3. Conjoint Analysis of Adrenocortical Carcinoma (ACC)
12.4. Key Market Forecast Assumptions
12.4.1. Cost Assumptions
12.4.2. Pricing Trends
12.4.3. Analogue Assessment
12.4.4. Launch Year and Therapy Uptakes
12.5. Total Market Size of Adrenocortical Carcinoma (ACC) in the 7MM
12.6. The United States
12.6.1. Total Market Size of Adrenocortical Carcinoma (ACC) in the United States
12.6.2. Market Size of Adrenocortical Carcinoma (ACC) by Therapies in the United States
12.7. EU4 and the UK
12.7.1. Total Market Size of Adrenocortical Carcinoma (ACC) in EU4 and the UK
12.7.2. Market Size of Adrenocortical Carcinoma (ACC) by Therapies in EU4 and the UK
12.8. Japan
12.8.1. Total Market Size of Adrenocortical Carcinoma (ACC) in Japan
12.8.2. Market Size of Adrenocortical Carcinoma (ACC) by Therapies in Japan
13. Unmet Needs of Adrenocortical Carcinoma (ACC)
14. SWOT Analysis of Adrenocortical Carcinoma (ACC)
15. KOL Views of Adrenocortical Carcinoma (ACC)
15.1. Expert/KOL Interview Highlights
16. Market Access and Reimbursement of Adrenocortical Carcinoma (ACC)
16.1. The US
16.2. In EU4 and the UK
16.2.1. Germany
16.2.2. France
16.2.3. Italy
16.2.4. Spain
16.2.5. United Kingdom
16.3. Japan
16.4. Summary and Comparison of Market Access and Pricing Policy Developments in 2025
16.5. Market Access and Reimbursement of Adrenocortical Carcinoma (ACC) Therapies
17. Appendix
17.1. Bibliography
17.2. Report Methodology
18. Analyst's Capabilities
19. Disclaimer
20. About the Publisher
List of Tables [45]
List of Figures [38]
A selection of companies mentioned in this report includes, but is not limited to:
- Esteve Daiichi Sankyo Cytovation Orphagen Pharmaceuticals
For more information about this report visit
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