(Albany, US), November 11, 2026 – DelveInsight’s ‘Adrenocortical Carcinoma Market Insights, Epidemiology and Market Forecast – 2036’ report delivers an in-depth understanding of Adrenocortical Carcinoma (ACC), historical and forecasted epidemiology, as well as the adrenocortical carcinoma market trends in the United States, EU4 (Germany, Spain, Italy, and France) and the United Kingdom, and Japan.

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Key Takeaways from the Adrenocortical Carcinoma Market Report

• In October 2026, the CTIS recorded recruitment activity for Cytovation’s Phase II CLARITY trial of CY-101 (getacatetide) in advanced ACC, with recruitment commencing in Spain on September 28 and France on October 23. The multinational trial is planned across four countries and ten clinical sites, with approximately 41 participants.

• In October 2026, a Phase II trial evaluating cemiplimab as maintenance treatment for advanced ACC became active in Italy, with recruitment commencing on October 12, 2026.

• In October 2026, the ADIUVO-2 Phase III trial evaluating adjuvant mitotane-based treatment for high-risk ACC added the Netherlands as an additional participating country on October 16, 2026.

• In September 2026, Imperial College London and the University of Birmingham announced the launch of IMS Solutions, a spin-out commercializing steroid fingerprint technology. It secured £1.1 million in seed funding and a £2.1 million NIHR grant, and its planned ACCelerate™ urine-based test is designed to distinguish benign adrenal tumours from malignant ACC, targeting a UK commercial launch by 2029.

• In September 2026, European regulators authorized a Phase II trial investigating HIPEC combined with regional adrenalectomy for Stage II and Stage III ACC.

• In July 2026, Cancer Research UK, Cytovation, and the Norwegian Cancer Society announced the multinational Phase II CLARITY trial of CY-101 (getacatetide) in advanced ACC.

• In February 2026, a Phase II trial (NCT06900595) commenced to evaluate cabozantinib, with or without cemiplimab, in adolescents and adults with advanced ACC.

• In 2026, Daiichi Sankyo initiated regulatory and clinical development activities for DS-9051b in advanced or metastatic ACC, with a first-in-human Phase I trial (NCT07189403).

• According to DelveInsight’s analysis, the adrenocortical carcinoma treatment market size was found to be ~USD 23 million in the 7MM in 2025, with a projected growth rate of ~15% CAGR during 2026-2036.

• Among the 7MM, the US accounted for the largest adrenocortical carcinoma market size, at ~USD 10 million in 2025, and the US adrenocortical carcinoma treatment market is projected to grow at a significant CAGR through 2036.

• In the US, mitotane (LYSODREN) is the only US FDA-approved drug specifically for the treatment of ACC, used both as an adjuvant therapy after surgery and for advanced, inoperable cases.

• The United States accounted for the highest incident cases of ACC, representing approximately 30% of total cases in the 7MM in 2025.

• ACC is frequently diagnosed at an advanced stage, and the absence of reliable screening tools for early detection remains a critical unmet need, delaying identification and worsening patient outcomes.

• The leading Adrenocortical Carcinoma Companies include Esteve, Daiichi Sankyo, Cytovation, Orphagen Pharmaceuticals, and others.

• Promising Adrenocortical Carcinoma Therapies include Mitotane (LYSODREN), DS-9051b, CY-101/Getacatetide, OR-449, and others.

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Adrenocortical Carcinoma Overview

Adrenocortical Carcinoma (ACC) is a rare and aggressive malignancy arising from the adrenal cortex. It can occur at any age but shows a bimodal distribution, affecting young children and adults in their 40s–50s. ACC may be functioning (hormone-secreting) or non-functioning, and functioning tumors often produce excess cortisol, androgens, or aldosterone, leading to clinical syndromes such as Cushing’s syndrome or virilization. Its etiology remains largely elusive, although genetic mutations and hereditary syndromes are implicated. The ENSAT (European Network for the Study of Adrenal Tumors) staging system is widely used to classify ACC and guide treatment decisions, ranging from Stage I (localized tumors ≤5 cm) to Stage IV (metastatic disease). Diagnosis involves a combination of hormonal workup (cortisol, DHEA-S, and aldosterone levels) and imaging studies such as contrast-enhanced CT or MRI, with PET scans assisting in detecting metastases. Histopathological confirmation following surgical resection or biopsy remains the gold standard, with criteria such as the Weiss score used to distinguish malignant from benign adrenal tumors. Because of its aggressive nature, ACC is often diagnosed at an advanced stage, contributing to poor prognosis and high recurrence rates.

Adrenocortical Carcinoma Epidemiology Segmentation in the 7MM

• Total Incident Cases of ACC

• Stage-specific Incident Cases of ACC

• Gender-specific Incident Cases of ACC

• Age-specific Incident Cases of ACC

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Adrenocortical Carcinoma Epidemiology Key Findings

• In the 7MM, Stage IV accounted for the highest share of ACC cases at ~35%, followed by Stage II, Stage III, and Stage I, which was the lowest at ~10%. This predominance may stem from delayed diagnosis, aggressive disease behavior, and limited screening methods.

• The incidence of ACC is higher in females (~60%) compared to males, indicating a notable gender disparity in disease occurrence.

• The United States accounted for approximately 30% of total ACC cases in the 7MM in 2025.

• The incidence of ACC increases with age, with the highest proportion observed in individuals aged >60 years, followed closely by those aged 40–60 years.

Approved Therapies for Adrenocortical Carcinoma

Mitotane (LYSODREN), distributed by Esteve, is an oral adrenolytic anticancer agent used primarily for the treatment of ACC. It works by selectively destroying adrenal cortical cells and inhibiting steroid hormone synthesis, thereby reducing cortisol production and tumor-related hormone excess. It is considered a cornerstone in both palliative and adjuvant treatment settings and is commonly used either alone or in combination. While mitotane monotherapy controls disease in select cases, combination therapies yield superior outcomes in aggressive or high-burden disease. Mitotane was marketed in the US in 1970 and in the EU in 2004.

Adrenocortical Carcinoma Pipeline Analysis

• CY-101/Getacatetide: Cytovation

Getacatetide is a membranolytic inhibitor of the Wnt/β-catenin pathway that helps the immune system fight cancer by exposing tumor neoantigens and inhibiting mechanisms that drive tumor growth and immune evasion. It has demonstrated early signs of antitumor activity in the Phase I (CICILIA) trial, particularly in tumors with dysregulated Wnt/β-catenin signaling. CY-101 is granted ODD in the US for the treatment of ACC, and the MHRA and Health Research Authority have approved the clinical trial application for the Phase II study in ACC.

• OR-449: Orphagen Pharmaceuticals

OR-449 is a first-in-class, orally bioavailable antagonist of steroidogenic factor-1 (SF-1, NR5A1), an orphan nuclear receptor and transcription factor essential for the growth and development of the adrenal gland. It is being developed for both adult and pediatric forms of ACC, and the US FDA has granted it a Rare Pediatric Disease Designation (RPDD).

• DS-9051b: Daiichi Sankyo

An oral ubiquitin protein ligase degrader in Phase I development for ACC, introducing another investigational candidate into the ACC pipeline.

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Adrenocortical Carcinoma Market Outlook

The standard of care for the adrenocortical carcinoma drugs market has historically relied on surgery for localized disease and systemic therapy for advanced or metastatic cases. The only widely approved drug specifically for ACC is mitotane (LYSODREN), used in both the adjuvant setting and in advanced disease, and the most commonly used regimen for advanced ACC is the EDP-M protocol, which combines etoposide, doxorubicin, and cisplatin with mitotane. The pipeline for ACC remains in early development, with investigational agents such as CY-101, DS-9051b, and OR-449 representing diverse approaches, including immunotherapy, targeted molecular inhibition, and modulation of steroid biosynthesis pathways. However, many programs are no longer actively progressing, with several trials primarily driven by academic institutions, and some studies, such as EO2401 in combination with nivolumab, have been discontinued. Overall, the launch of novel therapies, improved diagnostic techniques, and rising awareness are projected to propel steady growth in the adrenal cortex neoplasms market across the 7MM from 2022 to 2036, yielding significant commercial opportunities for existing products and pipeline candidates.

Key Factors Driving the Adrenocortical Carcinoma Market

• Increasing Diagnosis and Awareness: Improved imaging techniques and hormonal testing are enabling earlier and more accurate detection of ACC, and greater clinical awareness is expanding the treatable patient population and driving demand for therapies.

• Growing Pipeline and Research Activity: Ongoing research into immunotherapy and targeted therapies is expanding the pipeline, and increased R&D investment is expected to drive future market growth.

• High Unmet Medical Need: ACC is an aggressive cancer with poor prognosis and high recurrence rates, and limited treatment options create strong demand for effective therapies.

Adrenocortical Carcinoma Treatment Landscape

Treatment of ACC depends on disease stage and resectability. Surgical resection is the primary and only potentially curative treatment for localized disease. In advanced or metastatic cases, systemic therapy is required, with mitotane (LYSODREN) serving as the cornerstone adrenolytic agent, used alone or in combination with cytotoxic chemotherapy (EDP regimen: etoposide, doxorubicin, cisplatin). Radiation therapy is used for local control and management of recurrence. Despite these options, prognosis remains limited, and ongoing research into targeted therapies and immunotherapies is focused on improving long-term outcomes. Key unmet needs include limited treatment options, poor prognosis and high recurrence rates, the scarcity of large clinical trials in a rare disease, and the need for diagnostic and prognostic markers.

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Scope of the Adrenocortical Carcinoma Market Report

• Coverage: 7MM

• Study Period: 2022-2036

• Historical Year: 2022-2025

• Base Year: 2026

• Forecast Period: 2026-2036

• Adrenocortical Carcinoma Companies: Esteve, Daiichi Sankyo, Cytovation, Orphagen Pharmaceuticals, and others.

• Adrenocortical Carcinoma Therapies: Mitotane (LYSODREN), DS-9051b, CY-101/Getacatetide, OR-449, and others.

• Adrenocortical Carcinoma Market Dynamics: market drivers and market barriers

• Adrenocortical Carcinoma Competitive Intelligence Analysis: SWOT Analysis, Conjoint Analysis

• Adrenocortical Carcinoma Unmet Needs, KOL Views, Reimbursement, Cost Assumptions and Pricing Analogues

Table of Contents

1. Key Insights

2. Report Introduction

3. Executive Summary

4. Key Events

5. Epidemiology and Market Methodology of Adrenocortical Carcinoma (ACC)

6. Adrenocortical Carcinoma (ACC) Market Overview at a Glance

7. Disease Background and Overview of Adrenocortical Carcinoma (ACC)

8. Epidemiology and Patient Population of Adrenocortical Carcinoma (ACC)

9. Patient Journey of Adrenocortical Carcinoma (ACC)

10. Marketed Therapies

11. Emerging Therapies

12. Adrenocortical Carcinoma (ACC): Seven Major Market Analysis

13. Unmet Needs of Adrenocortical Carcinoma (ACC)

14. SWOT Analysis of Adrenocortical Carcinoma (ACC)

15. KOL Views of Adrenocortical Carcinoma (ACC)

16. Market Access and Reimbursement of Adrenocortical Carcinoma (ACC)

17. Appendix

18. DelveInsight Capabilities

19. Disclaimer

20. About DelveInsight

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