Adrenocortical Carcinoma - Market Insight, Epidemiology And Market Forecast - 2034

Adrenocortical Carcinoma - Market Insight, Epidemiology And Market Forecast - 2034



Key Highlights
  • The market size for ACC in the 7MM is anticipated to grow at a significant CAGR throughout the forecast period (2024–2034). This growth is attributed to the rising number of incident cases, the launch of emerging therapies, and increased awareness of ACC.
  • DelveInsight’s analyst forecasts that around 27% of all Adrenocortical Carcinoma (ACC) cases in the 7MM originate from the United States. Based on our calculations, the EU4 and the UK together saw approximately six hundred incident cases of Adrenocortical Carcinoma (ACC) in 2023.
  • In the 7MM, the market mainly consisted of Adjuvant therapy (Mitotane ± Strepto), which generated nearly USD 3.69 million in 2023.
  • The total market size of the Adrenocortical Carcinoma (ACC) treatment market is anticipated to experience growth during the forecast period due to the emergence of new and effective treatments, namely, EO2401 + Nivolumab, and others.
DelveInsight’s “Adrenocortical Carcinoma (ACC) – Market Insights, Epidemiology, and Market Forecast – 2034” report delivers an in-depth understanding of the Adrenocortical Carcinoma (ACC), historical and forecasted epidemiology and the Adrenocortical Carcinoma (ACC) market trends in the United States, EU4 (Germany, France, Italy, and Spain) and the United Kingdom, and Japan.

The Adrenocortical Carcinoma (ACC) market report provides current treatment practices, emerging drugs, market share of individual therapies, and current and forecasted 7MM Adrenocortical Carcinoma (ACC) market size from 2020 to 2034. The report also covers current Adrenocortical Carcinoma (ACC) treatment practices/algorithms and unmet medical needs to curate the best of the opportunities and assesses the underlying potential of the market.

Geography Covered
  • The United States
  • EU4 (Germany, France, Italy, and Spain) and the United Kingdom
  • Japan
Study Period: 2020–2034

Disease Understanding and Treatment Algorithm

Adrenocortical Carcinoma (ACC) Overview

ACC is a rare and aggressive cancer originating in the adrenal cortex above the kidneys. Risk factors include genetic syndromes like Li–Fraumeni and Beckwith–Wiedemann, inherited mutations, family history of adrenal tumors, and radiation exposure.

ACC progresses through stages based on tumor size and metastasis. Early stages involve confined tumors, while later stages may see invasion into nearby tissues or distant spread to organs like the liver, lungs, or bones. Staging guides treatment strategies by assessing disease extent.

Adrenocortical Carcinoma (ACC) Diagnosis

ACC diagnosis entails a comprehensive approach, starting with medical history review and physical examination to detect adrenal gland abnormalities. Imaging tests like CT, MRI, and PET scans visualize tumors and their extent, while blood and urine tests measure hormone levels and metabolic markers. Biopsies, though definitive, carry risk and aren't always performed. Genetic testing may be advised, particularly with familial history. Staging scans inform treatment decisions, often involving surgery, chemotherapy, radiation, or targeted therapy, supported by multidisciplinary healthcare teams.

Adrenocortical Carcinoma (ACC) Treatment

Treatment options for adrenocortical carcinoma (ACC) include surgery, radiation therapy, chemotherapy, and ongoing investigation into new treatments like immunotherapy and targeted therapy through clinical trials. Surgery, particularly adrenalectomy, is common for removing affected adrenal glands, potentially along with nearby tissues. Radiation therapy uses high-energy x-rays to kill cancer cells, while chemotherapy halts their growth with drugs. Immunotherapy engages the patient's immune system, and targeted therapy attacks specific cancer cells.

Adrenocortical Carcinoma (ACC) Epidemiology

As the market is derived using the patient-based model, the Adrenocortical Carcinoma (ACC) epidemiology chapter in the report provides historical as well as forecasted epidemiology segmented by Incident Cases of ACC, Stage-specific Incident Cases of ACC, Gender-specific Incident Cases of ACC, and Age-specific Incident Cases of ACC in the 7MM covering the United States, EU4 countries (Germany, France, Italy, and Spain) and the United Kingdom, and Japan, from 2020 to 2034.

As per DelveInsight’s estimations, the total incident cases of ACC in the 7MM were approximately 12 hundred in 2023 and are projected to increase during the forecast period, owing to the Increasing incidence trends over decades and advancements in diagnostic tools contribute to a better understanding and identification of this rare malignancy.
  • The total incident cases of ACC in the United States was approximately three hundred in 2023, and it is expected to increase at an estimated CAGR throughout the study period (2020-2034).
  • Among the 7MM, EU4 and the UK accounted for nearly six hundred incident cases of ACC, and these cases are expected to increase during the forecast period (2023-2034).
  • Among EU4 and the UK, Germany had the highest incident population of Adrenocortical Carcinoma (ACC), with ~160 cases in 2023. On the other hand, Spain had the lowest incident population in EU4 and the UK in 2023.
  • In Japan, there were around two hundred incident cases of ACC in 2023. These cases are expected to increase at a significant CAGR.
  • Stage-specific Incident Cases of ACC are segmented into four distinct stages. In the US, the distribution of ACC cases across stages reveals that Stage III has the highest cases at 38%, followed by Stage IV at 27%, Stage II at 20%, and Stage I at 15%.
  • The gender-specific incident ACC cases within the 7MM indicated an approximate distribution of 40% in males and 60% in females.
  • The highest proportion of ACC cases was estimated in the 41–60 years (53%) in EU4 and the UK, while the least cases were in the age group <20 years (3%).
Adrenocortical Carcinoma (ACC) Drug Chapters

The drug chapter segment of the Adrenocortical Carcinoma (ACC) report encloses a detailed analysis of Adrenocortical Carcinoma (ACC) marketed drugs and late-stage (Phase III and Phase II) pipeline drugs. It also understands Adrenocortical Carcinoma (ACC) clinical trial details, expressive pharmacological action, agreements and collaborations, approval, and patent details, advantages and disadvantages of each included drug, and the latest news and press releases.

Marketed Drugs

LYSODREN (Mitotane): HRA Pharma/Bristol-Myers Squibb

The adrenal cytotoxic agent mitotane (LYSODREN) has a central role in the systemic treatment of adrenocortical carcinoma (ACC), a rare and aggressive cancer of the adrenal glands. Mitotane has been evaluated and used for more than 60 years and, to date, is the only drug specifically approved for the treatment of ACC. Although ACC continues to be associated with a poor prognosis, mitotane has been shown to provide clinically significant benefit in a good proportion of ACC patients treated with the drug, both in advanced disease and adjuvant therapy settings. Approved in 1970 in the US and 2004 in the European Union, LYSODREN is listed among the ‘Off-Patent, Off-Exclusivity Drugs without an Approved Generic’ as outlined in the Orange Book.

Note: Detailed marketed therapies assessment will be provided in the final report of Adrenocortical Carcinoma (ACC).

Emerging Drugs

EO2401 + Nivolumab: Enterome/Bristol-Myers Squibb

EO2401, a novel immunotherapy developed by Enterome from their proprietary OncoMimics platform, consists of three synthetic peptides mimicking human Tumor-Associated Antigens: IL13Ra2, BIRC5/Survivin, and FOXM1. These peptides, derived from bacteria, target aggressive cancers like glioblastoma and adrenal tumors with minimal impact on healthy tissues. Nivolumab, an immune checkpoint inhibitor, aids T cell expansion and infiltration into tumors.

Note: Detailed emerging therapies assessment will be provided in the final report of Adrenocortical Carcinoma (ACC).

Adrenocortical Carcinoma (ACC) Market Outlook

Adrenocortical Carcinoma (ACC) has a diverse treatment classification associated with the disease landscape. The management of Adrenocortical Carcinoma (ACC) primarily revolves around the utilization of Adjuvant therapy (Mitotane ± Strepto) as needed.

The market for Adrenocortical Carcinoma (ACC) is expected to experience positive growth with the approval of potential drugs like EO2401 + Nivolumab, and others.
  • The Adrenocortical Carcinoma (ACC) market’s total size in the 7MM reached approximately USD 22.4 million in 2023. Projections indicate a substantial growth during the forecast period.
  • Out of the 7MM, the United States dominated the market in 2023, representing the largest share at nearly 47%.
  • In 2023, EU4 and the UK captured an estimated USD 8.2 million, which is anticipated to increase at a substantial CAGR. Among the European countries, Germany covered the largest market share in 2023, followed by the UK and France. Spain accounted for the least market in the same year.
  • Japan alone represented approximately 16% of the total Adrenocortical Carcinoma (ACC) market in 2023, projected to increase at a substantial CAGR during the study period.
  • The total market size of the Adrenocortical Carcinoma (ACC) treatment market is anticipated to experience growth during the forecast period due to the emergence of new and effective therapies.
Adrenocortical Carcinoma (ACC) Drugs Uptake

This section focuses on the uptake rate of potential drugs expected to launch in the market during 2020–2034. For example, EO2401 + Nivolumab in the US is expected to be launched by 2026, with a peak share of 60%.

Further detailed analysis of emerging therapies drug uptake in the report…

Adrenocortical Carcinoma (ACC) Pipeline Development Activities

The report provides insights into different therapeutic candidates in Phase III, Phase II, and Phase I stage. It also analyzes key players involved in developing targeted therapeutics.

Pipeline Development Activities

The report covers information on collaborations, acquisitions and mergers, licensing, and patent details for Adrenocortical Carcinoma (ACC) emerging therapies.

KOL Views

To keep up with current market trends, we take KOLs and SMEs’ opinions working in the domain through primary research to fill the data gaps and validate the secondary research. Industry Experts were contacted for insights on Adrenocortical Carcinoma (ACC) evolving treatment landscape, patient reliance on conventional therapies, patient therapy switching acceptability, and drug uptake along with challenges related to accessibility, including KOL from University of Michigan, Ann Arbor, MI, US, Interfaith Medical Center, Brooklyn, NY, US, Würzburg University Hospital, Würzburg, Germany, Center Léon-Bérard, Department of Medical Oncology, Lyon, France, University-Hospital of Padua, Padua, Italy, Catalan Institute of Oncology, Barcelona, Spain, University Hospital of Wales, Cardiff, United Kingdom, Cancer Institute Hospital of Japanese Foundation for Cancer Research, Tokyo, Japan, and others.

Delveinsight’s analysts connected with 50+ KOLs to gather insights; however, interviews were conducted with 15+ KOLs in the 7MM. Their opinion helps understand and validate current and emerging therapies, treatment patterns, or Adrenocortical Carcinoma (ACC) market trends. 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 and Conjoint Analysis. In the SWOT analysis, strengths, weaknesses, opportunities, and threats in terms of disease diagnosis, patient awareness, patient burden, competitive landscape, cost-effectiveness, and geographical accessibility of therapies are provided. These pointers are based on the Analyst’s discretion and assessment of the patient burden, cost analysis, and existing and evolving treatment landscape.

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.

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 route of administration, order of entry and designation, 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.

Market Access and Reimbursement

The high cost of therapies for the treatment is a major factor restraining the growth of the global drug market. Because of the high cost, the economic burden is increasing, leading the patient to escape from proper treatment.

The report further provides detailed insights on the country-wise accessibility and reimbursement scenarios, cost-effectiveness scenario of approved therapies, programs making accessibility easier and out-of-pocket costs more affordable, insights on patients insured under federal or state government prescription drug programs, etc.

Scope of the Report
  • The report covers a segment of key events, an executive summary, descriptive overview of Adrenocortical Carcinoma (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, disease progression, and treatment guidelines.
  • Additionally, an all-inclusive account of the current and emerging therapies and the elaborative profiles of late-stage and prominent therapies will impact the current treatment landscape.
  • A detailed review of the Adrenocortical Carcinoma (ACC) market, historical and forecasted market size, market share by therapies, detailed assumptions, and rationale behind the 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 shape and drive the 7MM Adrenocortical Carcinoma (ACC) market.
Adrenocortical Carcinoma (ACC) Report Insights
  • Patient Population
  • Therapeutic Approaches
  • Adrenocortical Carcinoma (ACC) Pipeline Analysis
  • Adrenocortical Carcinoma (ACC) Market Size and Trends
  • Existing and Future Market Opportunity
Adrenocortical Carcinoma (ACC) Report Key Strengths
  • 11 years Forecast
  • The 7MM Coverage
  • Adrenocortical Carcinoma (ACC) Epidemiology Segmentation
  • Key Cross Competition
  • Conjoint Analysis
  • Drugs Uptake and Key Market Forecast Assumptions
Adrenocortical Carcinoma (ACC) Report Assessment
  • Current Treatment Practices
  • Unmet Needs
  • Pipeline Product Profiles
  • Market Attractiveness
  • Qualitative Analysis (SWOT and Conjoint Analysis)
Key Questions

Market Insights
  • What was the Adrenocortical Carcinoma (ACC) total market size, the market size by therapies, and market share (%) distribution in 2020, and how would it all look in 2034? What are the contributing factors for this growth?
  • What unmet needs are associated with the current treatment market of Adrenocortical Carcinoma (ACC)?
  • What are the patents of emerging therapies for Adrenocortical Carcinoma (ACC)?
  • Which drug is going to be the largest contributor by 2034?
  • 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?
Epidemiology Insights
  • What are the disease risks, burdens, and unmet needs of Adrenocortical Carcinoma (ACC)? What will be the growth opportunities across the 7MM concerning the patient population of Adrenocortical Carcinoma (ACC)?
  • What is the historical and forecasted Adrenocortical Carcinoma (ACC) patient pool in the United States, EU4 (Germany, France, Italy, and Spain), the United Kingdom, and Japan?
  • Why do only limited patients appear for diagnosis?
  • Which country is more prevalent for Adrenocortical Carcinoma (ACC) and why?
  • What factors are affecting the diagnosis of the indication?
Current Treatment Scenario, Marketed Drugs, and Emerging Therapies
  • What are the current options for treating Adrenocortical Carcinoma (ACC)? What are the current guidelines for treating Adrenocortical Carcinoma (ACC) in the US and Europe?
  • How many companies are developing therapies for treating Adrenocortical Carcinoma (ACC)?
  • How many emerging therapies are in the mid-stage and late stage of development for treating Adrenocortical Carcinoma (ACC)?
  • What are the recent novel therapies, targets, mechanisms of action, and technologies developed to overcome the limitations of existing therapies?
  • What are the key designations that have been granted for the emerging therapies for Adrenocortical Carcinoma (ACC)?
  • Patient acceptability in terms of preferred treatment options as per real-world scenarios?
  • What are the country-specific accessibility issues of expensive, recently approved therapies? Focus on reimbursement policies.
  • What are the 7MM historical and forecasted markets of Adrenocortical Carcinoma (ACC)?
Reasons to Buy
  • The report will help develop business strategies by understanding the latest trends and changing treatment dynamics driving the Adrenocortical Carcinoma (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.
  • To understand the existing market opportunity 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.
  • Identifying strong upcoming players in the market will help devise strategies that will help get ahead of competitors.
  • Detailed analysis and ranking of potential current and emerging therapies under the conjoint analysis section to provide visibility around leading classes.
  • Highlights of Access and Reimbursement policies of approved therapies, barriers to accessibility of off-label expensive therapies, and patient assistance programs.
  • To understand the perspective of Key Opinion Leaders 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.
Frequently Asked Questions

1. What is the forecast period covered in the report?

The Adrenocortical Carcinoma (ACC) Epidemiology and Market Insight report for the 7MM covers the forecast period from 2023 to 2034, providing a projection of market dynamics and trends during this timeframe.

2. Who are the key players in the Adrenocortical Carcinoma (ACC) market?

The Adrenocortical Carcinoma (ACC) market is quite robust. The major players are Enterome, Corcept Therapeutics, Orphagen Pharmaceuticals, and others which are currently developing drugs for the treatment of Adrenocortical Carcinoma (ACC).

3. How is the market size estimated in the forecast report?

The market size is estimated through data analysis, statistical modeling, and expert opinions. It may consider factors such as incident cases, treatment costs, revenue generated, and market trends.

4. What is the key driver of the Adrenocortical Carcinoma (ACC) market?

The increase in the incident cases of Adrenocortical Carcinoma (ACC) and the launch of emerging therapies are attributed to be the key drivers for increasing Adrenocortical Carcinoma (ACC) market.

5. What is the expected impact of emerging therapies or advancements in Adrenocortical Carcinoma (ACC) treatment on the market?

Introducing new therapies, advancements in diagnostic techniques, and innovations in treatment approaches can significantly impact the Adrenocortical Carcinoma (ACC) treatment market. Market forecast reports may provide analysis and predictions regarding the potential impact of these developments.

6. Does the report provide insights into the competitive landscape of the market?

The market forecast report may include information on the competitive landscape, profiling key market players, their product offerings, partnerships, and strategies, and helping stakeholders understand the competitive dynamics of the Adrenocortical Carcinoma (ACC) market.


1. Key Insights
2. Report Introduction
3. Adrenocortical Carcinoma (ACC) Market Overview at a Glance
3.1. Market Share (%) Distribution of ACC in 2020 by Therapies
3.2. Market Share (%) Distribution of ACC in 2034 by Therapies
4. Epidemiology and Market Forecast Methodology
5. Executive Summary
6. Key Events
7. Disease Background and Overview
7.1. Introduction
7.2. Stages of ACC
7.3. Causes and Risk Factors
7.4. Pathophysiology
7.5. Symptoms and Clinical Presentations
7.6. Diagnosis
7.6.1. Diagnostic Algorithm
7.6.2. Diagnostic Guidelines
7.6.2.1. NCCN Guidelines for Patients: ACC
7.6.2.2. European Society of Endocrinology Clinical Practice Guidelines on the Management of Adrenocortical Carcinoma in Adults, in Collaboration With the European Network for the Study of Adrenal Tumors
7.6.2.3. ESMO–EURACAN Clinical Practice Guidelines for Diagnosis, Treatment and Follow-up of ACC
7.7. Treatment and Management
7.7.1. Treatment Algorithm
7.7.2. Treatment Guidelines
7.7.2.1. NCCN Guidelines for Patients: ACC
7.7.2.2. European Society of Endocrinology Clinical Practice Guidelines on the Management of Adrenocortical Carcinoma in Adults, in Collaboration With the European Network for the Study of Adrenal Tumors
7.7.2.3. ESMO–EURACAN Clinical Practice Guidelines for Diagnosis, Treatment, and Follow-up of ACC
8. Epidemiology and Patient Population
8.1. Key Findings
8.2. Assumptions and Rationale: The 7MM
8.2.1. Incident Cases of ACC
8.2.2. Stage-specific Incident Cases of ACC
8.2.3. Gender-specific Incident Cases of ACC
8.2.4. Age-specific Incident Cases of ACC
8.3. Total Incident Cases of ACC in the 7MM
8.4. The United States
8.4.1. Total Incident Cases of ACC in the US
8.4.2. Stage-specific Incident Cases of ACC in the US
8.4.3. Gender-specific Incident Cases of ACC in the US
8.4.4. Age-specific Incident Cases of ACC in the US
8.5. EU4 and the UK
8.5.1. Total Incident Cases of ACC in EU4 and the UK
8.5.2. Stage-specific Incident Cases of ACC in EU4 and the UK
8.5.3. Gender-specific Incident Cases of ACC in EU4 and the UK
8.5.4. Age-specific Cases of ACC in EU4 and the UK
8.6. Japan
8.6.1. Total Incident Cases of ACC in Japan
8.6.2. Stage-specific Incident Cases of ACC in Japan
8.6.3. Gender-specific Incident Cases of ACC in Japan
8.6.4. Age-specific Incident Cases of ACC in Japan
9. Patient Journey
10. Marketed Therapies
10.1. LYSODREN (Mitotane): HRA Pharma/Bristol-Myers Squibb
10.1.1. Product Description
10.1.2. Regulatory Milestone
10.1.3. Other Developmental Activities
11. Emerging Therapies
11.1. Key Cross Competition
11.2. EO2401 + Nivolumab: Enterome/Bristol-Myers Squibb
11.2.1. Product Description
11.2.2. Other Developmental Activities
11.2.3. Clinical Trials Information
11.2.4. Safety and Efficacy
11.2.5. Analysts’ Views
List to be continued in the report….
12. Adrenocortical Carcinoma (ACC): Market Analysis
12.1. Key Findings
12.2. Key Market Forecast Assumptions
12.3. Market Outlook
12.4. Total Market Size of ACC in the 7MM
12.5. Market Size of ACC by Therapies in the 7MM
12.6. Market Size of ACC in the United States
12.6.1. Total Market Size of ACC
12.6.2. Market Size of ACC by Therapies in the United States
12.7. Market Size of ACC in EU4 and the UK
12.7.1. Germany
12.7.1.1. Total Market Size of ACC
12.7.1.2. Market Size of ACC by Therapies in Germany
12.7.1. France
12.7.1.1. Total Market Size of ACC
12.7.1.2. Market Size of ACC by Therapies in France
12.7.1. Italy
12.7.1.1. Total Market Size of ACC
12.7.1.2. Market Size of ACC by Therapies in Italy
12.7.1. Spain
12.7.1.1. Total Market Size of ACC
12.7.1.2. Market Size of ACC by Therapies in Spain
12.7.1. The United Kingdom
12.7.1.1. Total Market Size of ACC
12.7.1.2. Market Size of ACC by Therapies in the UK
12.8. Market Size of ACC in Japan
12.8.1. Total Market Size of ACC
12.8.2. Market Size of ACC by Therapies in Japan
13. Key Opinion Leaders’ Views
14. SWOT Analysis
15. Unmet Needs
16. Market Access and Reimbursement
16.1. The United States
16.1.1. Center for Medicare and Medicaid Services (CMS)
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. The United Kingdom
16.3. Japan
16.3.1. MHLW
17. Appendix
17.1. Bibliography
17.2. Acronyms and Abbreviations
17.3. Report Methodology
18. DelveInsight Capabilities
19. Disclaimer
20. About DelveInsight

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