Adrenocortical Carcinoma Epidemiology
Adrenocortical Carcinoma (ACC) Insights and Trends
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According to DelveInsight’s analysis, the total incident cases of ACC were approximately 1,250 in the 7MM, including the United States, the EU4 (Germany, France, Italy, and Spain), the United Kingdom, and Japan) in 2025.
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ACC is a rare but aggressive malignancy arising from the adrenal cortex. Its etiology remains largely elusive, although genetic mutations and hereditary syndromes are implicated. Risk factors encompass age, gender disparities, and specific genetic predispositions.
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The oncocytic variant accounts for approximately 10% of adrenal cortical tumors and is the most common subtype, followed by the myxoid and sarcomatoid variants, which are both exceedingly rare. According to the 2022 WHO classification, oncocytic ACC is defined by the presence of oncocytic tumor cells comprising more than 90% of the tumor mass.
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ACC is a rare, aggressive cancer of the adrenal cortex, often producing excess hormones like cortisol, leading to syndromes like cushing’s.
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The ENSAT staging system is widely used to classify ACC and guide treatment decisions, with stages ranging from stage I to stage IV.
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Diagnosis of ACC involves a combination of hormonal evaluation and advanced imaging techniques to assess tumor functionality, size, and spread, with definitive confirmation achieved through histopathological analysis after surgical resection.
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A critical unmet need in ACC lies in establishing dependable screening methods for early detection. The majority of cases are diagnosed at advanced stages, hindering treatment options and prognoses. The absence of reliable screening tools delays identification, worsening patient outcomes.
Adrenocortical Carcinoma (ACC) Epidemiology Forecast in the 7MM
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2025 Incident Cases of Adrenocortical Carcinoma (ACC): 1,250
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2036 Projected Incident Cases of ACC: XXX
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ACC Growth Rate (2026–2036): ~0.1%
DelveInsight's ‘Adrenocortical Carcinoma Epidemiology Forecast – 2036’ report delivers an in-depth understanding of the ACC, historical and forecasted epidemiology, in the United States, EU4 (Germany, Spain, Italy, and France) and the United Kingdom, and Japan.
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Study Period
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2022–2036
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Historical Year
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2022–2025
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Forecast Period
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2026–2036
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Base Year
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2026
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Geographies Covered
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North America: The US;
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Europe: Germany, France, Italy, Spain, and the UK;
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Asia-Pacific: Japan
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Adrenocortical Carcinoma (ACC) Epidemiology CAGR
(Study period/Forecast period)
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~0.1% (2026–2036)
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Adrenocortical Carcinoma (ACC) Epidemiology Segmentation Analysis
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Patient Burden Assessment
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Total Incident Cases of ACC
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Stage-specific Incident Cases of ACC
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Gender-specific Incident Cases of ACC
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Age-specific Incident Cases of ACC
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Adrenocortical Carcinoma (ACC) Understanding and Diagnosis Algorithm
Adrenocortical Carcinoma (ACC) Overview
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. Functioning tumors often produce excess cortisol, androgens, or aldosterone, leading to clinical syndromes such as cushing’s syndrome or virilization. Due to its aggressive nature, ACC is frequently diagnosed at an advanced stage, contributing to poor prognosis and high recurrence rates.
Further details are provided in the report.
Adrenocortical Carcinoma (ACC) Diagnosis
Diagnosis of ACC involves a combination of biochemical evaluation and imaging studies. Hormonal workup is essential to detect excess steroid production, including cortisol, DHEA-S, and aldosterone levels. Imaging techniques such as contrast-enhanced CT or MRI help assess tumor size, characteristics, and local invasion, while PET scans may assist 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. Early and accurate diagnosis is critical for improving outcomes.
Further details are provided in the report.
Adrenocortical Carcinoma (ACC) Epidemiology
Key Findings from Adrenocortical Carcinoma (ACC) Epidemiological Analysis and Forecast
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In the 7MM, the distribution of ACC cases across stages reveals that Stage IV has the highest cases at ~35%, followed by Stage II, Stage III, and Stage I at lowest; ~10%. The predominance of Stage IV ACC cases in the 7MM may stem from delayed diagnosis, aggressive disease behavior, and limited screening methods, highlighting the urgent need for improved detection strategies.
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The incidence of ACC is observed to be higher in females (~60%) compared to males, indicating a notable gender disparity in disease occurrence.
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The United States accounted for the highest incident cases of ACC, representing approximately 30% of total cases in the 7MM in 2025.
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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.
Scope of the Report
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The report covers a segment of a descriptive overview of ACC, explaining its causes, signs and symptoms, and pathogenesis.
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Comprehensive insight has been provided into the epidemiology segments and forecasts, the future growth potential of the diagnosis rate, and disease progression.
Report Insights
Adrenocortical Carcinoma (ACC) Patient Population Forecast
Report Key Strengths
FAQs
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What are the disease risks, burdens, and unmet needs of ACC? What will be the growth opportunities across the 7MM concerning the patient population with ACC?
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What is the historical and forecasted ACC patient pool in the US, EU4 (Germany, France, Italy, and Spain), the UK, and Japan?
Reasons to Buy
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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.
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To understand key opinion leaders’ perspectives around the diagnostic challenges to overcome barriers in the future.
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Detailed insights on various factors hampering disease diagnosis and other existing diagnostic challenges.
1 Key Insights
2 Report Introduction
3 Executive Summary
4 Epidemiology Methodology of Adrenocortical Carcinoma (ACC)
5 Adrenocortical Carcinoma (ACC) Epidemiology Overview at a Glance
5.1 Patient Share (%) Distribution by Country in 2025 in the 7MM
5.2 Patient Share (%) Distribution by Country in 2036 in the 7MM
6 Disease Background and Overview of Adrenocortical Carcinoma (ACC)
6.1 Introduction
6.2 Causes
6.3 Signs and Symptoms
6.4 Diagnosis
6.4.1 Differential Diagnosis
6.4.2 Diagnostic Algorithm
6.4.3 Diagnostic Guidelines
7 Epidemiology and Patient Population of Adrenocortical Carcinoma (ACC)
7.1 Key Findings
7.2 Assumptions and Rationale
7.3 Total Incident Cases of Adrenocortical Carcinoma (ACC) in the 7MM
7.4 The United States
7.4.1 Total Incident Cases of Adrenocortical Carcinoma (ACC) in the United States
7.4.2 Stage-specific Incident Cases of Adrenocortical Carcinoma (ACC) in the United States
7.4.3 Gender-specific Incident Cases of Adrenocortical Carcinoma (ACC) in the United States
7.4.4 Age-specific Incident Cases of Adrenocortical Carcinoma (ACC) in the United States
7.5 EU4 and the UK
7.5.1 Total Incident Cases of Adrenocortical Carcinoma (ACC) in EU4 and the UK
7.5.2 Stage-specific Incident Cases of Adrenocortical Carcinoma (ACC) in EU4 and the UK
7.5.3 Gender-specific Incident Cases of Adrenocortical Carcinoma (ACC) in EU4 and the UK
7.5.4 Age-specific Incident Cases of Adrenocortical Carcinoma (ACC) in EU4 and the UK
7.6 Japan
7.6.1 Total Incident Cases of Adrenocortical Carcinoma (ACC) in Japan
7.6.2 Stage-specific Incident Cases of Adrenocortical Carcinoma (ACC) in Japan
7.6.3 Gender-specific Incident Cases of Adrenocortical Carcinoma (ACC) in Japan
7.6.4 Age-specific Incident Cases of Adrenocortical Carcinoma (ACC) in Japan
8 Appendix
8.1 Bibliography
8.2 Report Methodology
9 DelveInsight Capabilities
10 Disclaimer
11 About DelveInsight
List of Tables:
Table 1 Summary of Adrenocortical Carcinoma Epidemiology (2021-2034)
Table 2 Stages of Adrenocortical Carcinoma (1/2)
Table 3 Stages of Adrenocortical Carcinoma (2/2)
Table 4 Adrenocortical Carcinoma: Surveillance Tests as Needed
Table 5 Diagnostic Work-up of (suspected) Adrenocortical Carcinoma
Table 6 Total Incident Cases of Adrenocortical Carcinoma in the 7MM (2021-2034)
Table 7 Total Incident Cases of Adrenocortical Carcinoma in the US (2021-2034)
Table 8 Stage-specific Incident Cases of Adrenocortical Carcinoma in the US (2021-2034)
Table 9 Gender-specific Incident Cases of Adrenocortical Carcinoma in the US (2021-2034)
Table 10 Age-specific Incident Cases of Adrenocortical Carcinoma in the US (2021-2034)
Table 11 Total Incident Cases of Adrenocortical Carcinoma in EU4 and the UK (2021-2034)
Table 12 Stage-specific Incident Cases of Adrenocortical Carcinoma in EU4 and the UK (2021-2034)
Table 13 Gender-specific Incident Cases of Adrenocortical Carcinoma in EU4 and the UK (2021-2034)
Table 14 Age-specific Incident Cases of Adrenocortical Carcinoma in EU4 and the UK (2021-2034)
Table 15 Total Incident Cases of Adrenocortical Carcinoma in Germany (2021-2034)
Table 16 Stage-specific Incident Cases of Adrenocortical Carcinoma in Germany (2021-2034)
Table 17 Gender-specific Incident Cases of Adrenocortical Carcinoma in Germany (2021-2034)
Table 18 Age-specific Incident Cases of Adrenocortical Carcinoma in Germany (2021-2034)
Table 19 Total Incident Cases of Adrenocortical Carcinoma in France (2021-2034)
Table 20 Stage-specific Incident Cases of Adrenocortical Carcinoma in France (2021-2034)
Table 21 Gender-specific Incident Cases of Adrenocortical Carcinoma in France (2021-2034)
Table 22 Age-specific Incident Cases of Adrenocortical Carcinoma in France (2021-2034)
Table 23 Total Incident Cases of Adrenocortical Carcinoma in Italy (2021-2034)
Table 24 Stage-specific Incident Cases of Adrenocortical Carcinoma in Italy (2021-2034)
Table 25 Gender-specific Incident Cases of Adrenocortical Carcinoma in Italy (2021-2034)
Table 26 Age-specific Incident Cases of Adrenocortical Carcinoma in Italy (2021-2034)
Table 27 Total Incident Cases of Adrenocortical Carcinoma in Spain (2021-2034)
Table 28 Stage-specific Incident Cases of Adrenocortical Carcinoma in Spain (2021-2034)
Table 29 Gender-specific Incident Cases of Adrenocortical Carcinoma in Spain (2021-2034)
Table 30 Age-specific Incident Cases of Adrenocortical Carcinoma in Spain (2021-2034)
Table 31 Total Incident Cases of Adrenocortical Carcinoma in the UK (2021-2034)
Table 32 Stage-specific Incident Cases of Adrenocortical Carcinoma in the UK (2021-2034)
Table 33 Gender-specific Incident Cases of Adrenocortical Carcinoma in the UK (2021-2034)
Table 34 Age-specific Incident Cases of Adrenocortical Carcinoma in the UK (2021-2034)
Table 35 Total Incident Cases of Adrenocortical Carcinoma in Japan (2021-2034)
Table 36 Stage-specific Incident Cases of Adrenocortical Carcinoma in Japan (2021-2034)
Table 37 Gender-specific Incident Cases of Adrenocortical Carcinoma in Japan (2021-2034)
Table 38 Age-specific Incident Cases of Adrenocortical Carcinoma in Japan (2021-2034)
List of Figures:
Figure 1 Adrenocortical Carcinoma (Adrenocortical Carcinoma)
Figure 2 Stages of Adrenocortical Carcinoma
Figure 3 Causes and Risk Factors of Adrenocortical Carcinoma
Figure 4 Pathogenesis of Adrenocortical Carcinoma
Figure 5 Symptoms of Adrenocortical Carcinoma
Figure 6 Symptoms of Functioning and Non-functioning Adrenocortical Carcinoma
Figure 7 Diagnostic Algorithm for Adrenocortical Carcinoma
Figure 8 Total Incident Cases of Adrenocortical Carcinoma in the 7MM (2021-2034)
Figure 9 Total Incident Cases of Adrenocortical Carcinoma in the US (2021-2034)
Figure 10 Stage-specific Incident Cases of Adrenocortical Carcinoma in the US (2021-2034)
Figure 11 Gender-specific Incident Cases of Adrenocortical Carcinoma in the US (2021-2034)
Figure 12 Age-specific Incident Cases of Adrenocortical Carcinoma in the US (2021-2034)
Figure 13 Total Incident Cases of Adrenocortical Carcinoma in EU4 and the UK (2021-2034)
Figure 14 Stage-specific Incident Cases of Adrenocortical Carcinoma in EU4 and the UK (2021-2034)
Figure 15 Gender-specific Incident Cases of Adrenocortical Carcinoma in EU4 and the UK (2021-2034)
Figure 16 Age-specific Incident Cases of Adrenocortical Carcinoma in EU4 and the UK (2021-2034)
Figure 17 Total Incident Cases of Adrenocortical Carcinoma in Germany (2021-2034)
Figure 18 Stage-specific Incident Cases of Adrenocortical Carcinoma in Germany (2021-2034)
Figure 19 Gender-specific Incident Cases of Adrenocortical Carcinoma in Germany (2021-2034)
Figure 20 Age-specific Incident Cases of Adrenocortical Carcinoma in Germany (2021-2034)
Figure 21 Total Incident Cases of Adrenocortical Carcinoma in France (2021-2034)
Figure 22 Stage-specific Incident Cases of Adrenocortical Carcinoma in France (2021-2034)
Figure 23 Gender-specific Incident Cases of Adrenocortical Carcinoma in France (2021-2034)
Figure 24 Age-specific Incident Cases of Adrenocortical Carcinoma in France (2021-2034)
Figure 25 Total Incident Cases of Adrenocortical Carcinoma in Italy (2021-2034)
Figure 26 Stage-specific Incident Cases of Adrenocortical Carcinoma in Italy (2021-2034)
Figure 27 Gender-specific Incident Cases of Adrenocortical Carcinoma in Italy (2021-2034)
Figure 28 Age-specific Incident Cases of Adrenocortical Carcinoma in Italy (2021-2034)
Figure 29 Total Incident Cases of Adrenocortical Carcinoma in Spain (2021-2034)
Figure 30 Stage-specific Incident Cases of Adrenocortical Carcinoma in Spain (2021-2034)
Figure 31 Gender-specific Incident Cases of Adrenocortical Carcinoma in Spain (2021-2034)
Figure 32 Age-specific Incident Cases of Adrenocortical Carcinoma in Spain (2021-2034)
Figure 33 Total Incident Cases of Adrenocortical Carcinoma in the UK (2021-2034)
Figure 34 Stage-specific Incident Cases of Adrenocortical Carcinoma in the UK (2021-2034)
Figure 35 Gender-specific Incident Cases of Adrenocortical Carcinoma in the UK (2021-2034)
Figure 36 Age-specific Incident Cases of Adrenocortical Carcinoma in the UK (2021-2034)
Figure 37 Total Incident Cases of Adrenocortical Carcinoma in Japan (2021-2034)
Figure 38 Stage-specific Incident Cases of Adrenocortical Carcinoma in Japan (2021-2034)
Figure 39 Gender-specific Incident Cases of Adrenocortical Carcinoma in Japan (2021-2034)
Figure 40 Age-specific Incident Cases of Adrenocortical Carcinoma in Japan (2021-2034)
Figure 41 Patient Journey of Adrenocortical Carcinoma