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

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      DelveInsight Business Research, LLP

      12 Jun, 2024, 21:31 GMT

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      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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      About DelveInsight

      DelveInsight is a leading Business Consultant and Market Research firm focused exclusively on life sciences. It supports pharma companies by providing comprehensive end-to-end solutions to improve their performance. Get hassle-free access to all the healthcare and pharma market research reports through our subscription-based platform PharmDelve.

      Contact Us
      Shruti Thakur
      info@delveinsight.com
      +14699457679
      www.delveinsight.com

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