RecruitingPhase 1NCT06487481
Oncology
Adrenocortical Carcinoma
Also known as ACC, adrenal cortex cancer, TP53/CTNNB1 adrenal carcinoma
Adrenocortical carcinoma is a rare, aggressive malignancy of the adrenal cortex with a highly variable prognosis ranging from curable resected stage I tumours to rapidly fatal stage IV disease with a median survival under 12 months. The mol
ORPHA:1501 ↗Gene TP53Gene CTNNB1Gene IGF2Prevalence 1–2 in 1,000,000Onset Bimodal: children under 5 and adults in 4th–5th decadeSporadic; Li-Fraumeni syndrome (germline TP53) in familial cases
24
studies recruiting now
as of 7 Sept 2026
115
studies registered in total
as of 7 Sept 2026
5
countries with a recruiting site
as of 7 Sept 2026
28 Mar 2025
most recent study posted
among recruiting studies
Recruiting trials
RecruitingPhase 2NCT05286814
PDS01ADC in Combination With Hepatic Artery Infusion Pump (HAIP) and Systemic Therapy for Subjects With Metastatic Colorectal Cancer, Intrahepatic Cholangiocarcinoma, or Metastatic Adrenocortical Carcinoma
RecruitingPhase 2NCT06900595
Testing the Addition of an Anti-Cancer Drug, Cabozantinib to the Immunotherapy Drug Cemiplimab (REGN2810), in Adolescents and Adults With Advanced Adrenocortical Cancer
RecruitingNCT00453674
German Adrenocortical Carcinoma Registry
RecruitingPhase 3NCT03583710
Mitotane With or Without Cisplatin and Etoposide After Surgery in Treating Patients With Stage I-III Adrenocortical Cancer With High Risk of Recurrence
Showing the 5 most recently updated recruiting studies, as recorded 7 Sept 2026. Live status on each study page.
See all 24 recruiting studiesWhere recruiting studies are running
Countries with at least one recruiting site among the studies above, 7 Sept 2026. Tap a country to search trials there.
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About Adrenocortical Carcinoma
Adrenocortical carcinoma is a rare, aggressive malignancy of the adrenal cortex with a highly variable prognosis ranging from curable resected stage I tumours to rapidly fatal stage IV disease with a median survival under 12 months. The molecular landscape is characterised by IGF2 overexpression, somatic TP53 mutations, CTNNB1 activating mutations, and alterations in the CDKN2A and RB1 pathways; germline TP53 mutations cause the Li-Fraumeni syndrome phenotype, particularly relevant in paediatric ACC where up to 80% of Brazilian paediatric patients carry the founder R337H TP53 variant. Mitotane, an adrenolytic drug, remains the cornerstone of adjuvant and palliative systemic therapy, though combination chemotherapy with etoposide, doxorubicin, cisplatin, and mitotane (EDP-M) is used for advanced disease.
Common clinical features
Symptoms of cortisol excess (Cushing's syndrome): weight gain, hypertension, diabetes, proximal muscle weakness, and striaeSymptoms of androgen excess: virilisation, hirsutism, and menstrual irregularity in womenAbdominal or flank pain and a palpable abdominal mass from a large tumourIncidental adrenal mass discovered on imaging for unrelated indicationsHypokalaemia and hypertension from aldosterone-secreting tumours (less common)Constitutional symptoms: weight loss, anorexia, and fatigue in advanced diseaseBack pain and bone pain from skeletal metastases
From Orphanet’s phenotype annotations (CC BY 4.0). Not a complete list.
Treatments being studied
1 approved treatment and 25 in clinical development, from Open Targets (CC BY 4.0). Not medical advice.
Approved: Mitotane (Lysodren)
Phase 3Cisplatin (Cisplatin)
Phase 3Etoposide (Etoposide)
Phase 3Linsitinib
Phase 2Gossypol Acetic Acid
Phase 2Antineoplaston A10
Phase 2Nivolumab (Nivolumab bms)
Phase 2Axitinib (Inlyta)
Phase 2Paclitaxel (Abraxane)
+ 17 more in development
Before you apply
Things trial teams commonly ask about for Adrenocortical Carcinoma. Not eligibility rules; those are set by each study.
- Hormonal secretory status — confirmed by 24-hour urinary steroid profile and plasma/urinary catecholamines — must be documented and managed before trial enrolment; uncontrolled hormone excess may be an exclusion criterion.
- Mitotane plasma levels (target 14–20 mg/L therapeutic range) and mitotane treatment history are important context for many trials; trials combining mitotane with investigational agents require stable mitotane levels before randomisation.
- Comprehensive molecular profiling (including CTNNB1, TP53, CDK4, and MSI status) is increasingly required for biomarker-selected trials — request next-generation sequencing of tumour tissue if not already performed.
Related conditions
Information, not medical advice. Trial listings are shown as recorded on ClinicalTrials.gov; whether any study is right for you is a decision for you and your clinicians, and eligibility is decided by each research team. Disease information from Orphanet (CC BY 4.0).