Adrenocortical Carcinoma Market to Exhibit Growth at a CAGR of 11.1% by 2034 | DelveInsight

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Jun 12, 2024, 17:31 ET

According to DelveInsight's' estimates, the adrenocortical carcinoma market in 7MM is expected to show positive growth, during the forecast period (2024–2034), mainly attributed to the anticipated launch of emerging therapies and the increasing cases of adrenocortical carcinoma.

LAS VEGAS, June 12, 2024 /PRNewswire/ -- DelveInsight's Adrenocortical Carcinoma Market Insights report includes a comprehensive understanding of current treatment practices, adrenocortical carcinoma emerging drugs, market share of individual therapies, and current and forecasted market size from 2020 to 2034, segmented into 7MM [the United States, the EU4 (Germany, France, Italy, and Spain) and the United Kingdom, and Japan].

Key Takeaways from the Adrenocortical Carcinoma Market Report

According to DelveInsight's analysis, the adrenocortical carcinoma market's total size in the 7MM reached approximately USD 22.4 million in 2023.
As per DelveInsight's estimations, the total incident cases of adrenocortical carcinoma in the 7MM were approximately 1,200 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.
Leading adrenocortical carcinoma companies such as Enterome, Bristol-Myers Squibb, Cytovation AS, Orphagen Pharmaceuticals, and others are developing novel Adrenocortical Carcinoma drugs that can be available in the adrenocortical carcinoma market in the coming years.
The promising adrenocortical carcinoma therapies in the pipeline include EO2401 + Nivolumab, CyPep-1, and OR-449, among others.
In January 2023, Orphagen Pharmaceuticals announced that the United States Food and Drug Administration (US FDA) has granted a rare pediatric disease designation (RPDD) to OR-449 for treating pediatric ACC.
ADC Therapeutics announced the oral presentation of ADCT-701 at the AACR 2024 conference which was titled as "Targeting DLK1, a Notch ligand, with an antibody-drug conjugate in adrenocortical carcinoma"

Discover which therapies are expected to grab the major adrenocortical carcinoma market share @ Adrenocortical Carcinoma Market Report

Adrenocortical Carcinoma Overview

Adrenocortical carcinoma is a rare but aggressive cancer originating in the adrenal cortex. The cause of adrenocortical carcinoma is largely unknown, though genetic mutations and hereditary conditions are believed to play a role. Risk factors include age, gender differences, and specific genetic tendencies. Symptoms of adrenocortical carcinoma include abdominal pain, unexplained weight loss, hormonal imbalances, and detectable abdominal masses. Due to its rarity and vague symptoms, early detection is often difficult. Adrenocortical carcinoma progresses through four stages. Stages I and II are characterized by localized disease, Stage III involves lymph node involvement, and Stage IV indicates advanced disease with metastasis to distant organs.

Diagnosing adrenocortical carcinoma involves a thorough process beginning with a review of medical history and a physical examination to identify abnormalities in the adrenal gland. Imaging tests such as CT, MRI, and PET scans are used to visualize tumors and assess their spread. Blood and urine tests are conducted to measure hormone levels and metabolic markers. Although biopsies provide a definitive diagnosis, they come with risks and are not always performed. Genetic testing might be recommended, especially if there is a family history of the disease. Staging scans help guide treatment options, which typically include surgery, chemotherapy, radiation, or targeted therapy, all coordinated by multidisciplinary healthcare teams.

Adrenocortical Carcinoma Epidemiology Segmentation

DelveInsight's analyst forecasts that around 27% of all adrenocortical carcinoma cases in the 7MM originate from the United States. Based on our calculations, the EU4 and the UK together saw ~600 incident cases of adrenocortical carcinoma (ACC) in 2023.

The adrenocortical carcinoma market report proffers epidemiological analysis for the study period 2020–2034 in the 7MM segmented into:

Adrenocortical Carcinoma Incident Cases
Adrenocortical Carcinoma Stage-specific Incident Cases
Adrenocortical Carcinoma Gender-specific Incident Cases
Adrenocortical Carcinoma Age-specific Incident Cases

Adrenocortical Carcinoma Treatment Market

First-line therapies for adrenocortical carcinoma involve a personalized approach tailored to patient-specific prognostic factors. Treatment strategies vary based on these factors, incorporating both local and systemic methods. Patients with good performance status and aggressive disease may undergo combination therapy, such as EDP + M, which includes etoposide, doxorubicin, cisplatin, and mitotane. In contrast, those with less aggressive disease might receive mitotane monotherapy, sometimes supplemented with locoregional therapies.

Despite the effectiveness of EDP-M as a first-line therapy for adrenocortical carcinoma, patient prognosis remains poor. Second-line treatments, such as streptozocin plus mitotane (S+M) and gemcitabine plus capecitabine (G+C), show limited response rates (around 10%). Although G+C is considered modestly effective, it lacks significant predictive molecular markers but generally has tolerable side effects.

Temozolomide used as a third-line chemotherapy for advanced adrenocortical carcinoma, demonstrates anti-tumor activity in vitro. While some clinical studies show disease control in certain patients, its overall efficacy is limited. Ongoing research seeks to define temozolomide's optimal role, especially in identifying patient subsets that may respond well to treatment.

Future advances in adrenocortical carcinoma treatment may utilize new chemotherapeutic agents, vascular growth inhibitors, and small-molecule therapies, driven by a better understanding of the molecular pathways involved in tumorigenesis. These emerging strategies hold promise for improving therapeutic efficacy and patient outcomes.

Learn more about the FDA-approved drugs for adrenocortical carcinoma @ Drugs for Adrenocortical Carcinoma Treatment

Adrenocortical Carcinoma Pipeline Therapies and Key Companies

The lack of research and development efforts in adrenocortical carcinoma is due to its categorization as an ultra rare disease, indicating a limited pipeline with only a handful of therapies in Phase II, Phase I/II, and preclinical investigations. With only one double combination therapy, "EO2401 + Nivolumab," showing promise in emerging in the ACC landscape, the field faces considerable challenges. However, this underscores the urgent need for increased investment and focus to propel adrenocortical carcinoma treatment strategies forward.

EO2401, an innovative therapeutic vaccine derived from the microbiome and developed by Enterome, is designed to combat solid tumors such as glioblastoma and adrenocortical carcinoma. By utilizing specific peptides that mimic antigens associated with tumors and triggering immune responses, it seeks to instigate a focused attack by memory T cells against tumor cells. Clinical trials, particularly in cases of glioblastoma, have demonstrated robust immune responses, encouraging efficacy, and a favorable safety profile, especially when combined with checkpoint inhibitors. EO2401 is also exhibiting promise in trials for adrenocortical carcinoma.

Enterome is currently assessing EO2401 in a clinical trial for treating adrenal tumors (SPENCER study, EOADR1-19). In March 2021, Enterome entered into a clinical trial collaboration and supply agreement with Bristol Myers Squibb (BMS) to support EO2401, an OncoMimics vaccine in clinical development. Under this agreement, BMS has committed to providing nivolumab at no cost for patients participating in the combined therapy clinical trial SPENCER (EOADR1-19).

CyPep-1 and OR-449 are two promising pharmaceutical candidates currently progressing through clinical development for adrenocortical carcinoma. CyPep-1, a synthetic peptide targeting tumor membranes with 27-D-amino acids in an alpha-helical structure, is being developed by Cytovation AS. It is set to begin Phase II clinical trials in the first half of 2024 as a standalone treatment for adrenocortical carcinoma.

On the other hand, OR-449 from adrenocortical carcinoma is a novel preclinical compound designed as an oral antagonist targeting the orphan nuclear receptor steroidogenic factor-1 (SF-1 or NR5A1). Notably, OR-449 has received a Rare Pediatric Disease Designation (RPDD) from the FDA. Consequently, the company plans to submit an IND application to the FDA later this year, marking the initiation of Phase I clinical trials. These innovative therapies, projected to become available in the therapeutic market between 2024 and 2034, are poised to significantly advance the treatment options for adrenocortical carcinoma.

Discover more about adrenocortical carcinoma drugs in development @ Adrenocortical Carcinoma Clinical Trials

Adrenocortical Carcinoma Market Dynamics

The dynamics of the adrenocortical carcinoma market are expected to change in the coming years. The availability of surgical interventions like adrenalectomy, along with the exclusive approval of Mitotane, consolidating its position as the primary therapeutic option for adrenocortical carcinoma, and the potential of the EO2401 + Nivolumab combination to address immunotherapy resistance, presents a significant opportunity in the scarce therapeutic market of adrenocortical carcinoma, enhancing treatment accessibility and inviting key market players to enter.

Furthermore, many potential therapies are being investigated for the treatment of adrenocortical carcinoma, and it is safe to predict that the treatment space will significantly impact the adrenocortical carcinoma market during the forecast period. Moreover, the anticipated introduction of emerging therapies with improved efficacy and a further improvement in the diagnosis rate are expected to drive the growth of the adrenocortical carcinoma market in the 7MM.

However several factors may impede the growth of the adrenocortical carcinoma market. The limited availability of systemic therapies, coupled with surgery as the primary treatment, poses a weakness due to restricted treatment options for adrenocortical carcinoma, exacerbated by the rarity of the disease, hindering large-scale clinical trials and challenging evidence-based treatment guidelines. Furthermore, resistance or ineffective responses to existing adrenocortical carcinoma treatment options, like mitotane monotherapy, lead to unfavorable patient outcomes, posing a critical challenge, further compounded by the limited availability of funding and resources for research and development due to adrenocortical carcinoma's ultra-rare status, which poses a significant threat.

Moreover, adrenocortical carcinoma treatment poses a significant economic burden and disrupts patients' overall well-being and QOL. Furthermore, the adrenocortical carcinoma market growth may be offset by failures and discontinuation of emerging therapies, unaffordable pricing, market access and reimbursement issues, and a shortage of healthcare specialists. In addition, the undiagnosed, unreported cases and the unawareness about the disease may also impact the adrenocortical carcinoma market growth.

Adrenocortical Carcinoma Market Report Metrics	Details
Study Period	2020–2034
Coverage	7MM [the United States, the EU4 (Germany, France, Italy, and Spain) and the United Kingdom, and Japan].
Adrenocortical Carcinoma Market CAGR
Adrenocortical Carcinoma Market Size in 2023	USD 22.4 Million
Key Adrenocortical Carcinoma Companies	Enterome, Bristol-Myers Squibb, Cytovation AS, Orphagen Pharmaceuticals, and others
Key Pipeline Adrenocortical Carcinoma Therapies	EO2401 + Nivolumab, CyPep-1, OR-449, and others

Scope of the Adrenocortical Carcinoma Market Report

Therapeutic Assessment: Adrenocortical Carcinoma current marketed and emerging therapies
Adrenocortical Carcinoma Market Dynamics: Key Market Forecast Assumptions of Emerging Adrenocortical Carcinoma Drugs and Market Outlook
Competitive Intelligence Analysis: SWOT analysis and Market entry strategies
Unmet Needs, KOL's views, Analyst's views, Adrenocortical Carcinoma Market Access and Reimbursement

To know more about adrenocortical carcinoma treatment guidelines, visit @ Adrenocortical Carcinoma Management

Table of Contents

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
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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