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Adrenocortical Carcinoma (ACC) Clinical Trials

Also called ACC, adrenal cortex cancer, adrenal cancer, adrenal gland cancer

Adrenocortical carcinoma (ACC) is a rare endocrine malignancy arising from the cortex (outer layer) of the adrenal glands. The adrenal cortex produces steroid hormones essential for metabolism, blood pressure, and sexual development.

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About Adrenocortical Carcinoma

Adrenocortical carcinoma (ACC) is a rare endocrine malignancy arising from the cortex (outer layer) of the adrenal glands. The adrenal cortex produces steroid hormones essential for metabolism, blood pressure, and sexual development. ACC tumors may overproduce these hormones, causing Cushing syndrome (excess cortisol), virilization (excess androgens), feminization (excess estrogen), or hyperaldosteronism.

About 50-60% of ACCs are hormonally active, which can lead to earlier diagnosis through hormonal symptoms. Non-functioning tumors are often found incidentally on imaging or when they become large enough to cause pain. Complete surgical resection is the only curative treatment and is the cornerstone of management. For unresectable or metastatic disease, mitotane (an adrenolytic drug that destroys adrenal tissue) combined with etoposide, doxorubicin, and cisplatin (EDP-M) is the standard systemic approach. Prognosis depends heavily on stage at diagnosis and completeness of surgical resection.

Common Symptoms of Adrenocortical Carcinoma

Recognizing the signs of Adrenocortical Carcinoma early can lead to faster diagnosis and better outcomes. Symptoms may vary in severity from person to person. If you or a loved one are experiencing any of the following, consider speaking with a specialist.

  • Rapid, unexplained weight gain (especially in the face and trunk)
  • Excess hair growth or deepening voice in women (from androgen overproduction)
  • High blood pressure resistant to medication
  • Muscle weakness and easy bruising
  • Abdominal pain or a palpable mass
  • Irregular menstrual periods or breast enlargement in men

Who Adrenocortical Carcinoma Affects

Has a bimodal age distribution: children under 5 and adults ages 40-50. Slightly more common in women. Associated with Li-Fraumeni syndrome (TP53 mutations), Beckwith-Wiedemann syndrome, and MEN1. Higher incidence in southern Brazil due to a founder TP53 mutation.

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FDA-Approved Treatments for Adrenocortical Carcinoma

There is currently 1 FDA-approved medication for Adrenocortical Carcinoma. These therapies represent the current standard of care and may be used alongside or compared against investigational treatments in active clinical trials.

mitotane
Esteve Pharmaceuticals (formerly HRA Pharma Rare Diseases)
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Source: openFDA drug labeling data. This list may not include all treatments. Always consult your doctor.

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Side Effect Explorer

Real-world side effect reports from the FDA Adverse Event Reporting System (FAERS). Includes both FDA-approved drugs and investigational therapies from active clinical trials. Click any drug to see what patients reported.

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Genetic Testing

Genetic testing can confirm a diagnosis, guide treatment decisions, and identify family members who may be at risk.

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Trusted Adrenocortical Carcinoma Resources

Reputable organizations and medical references for learning more about Adrenocortical Carcinoma, including disease registries, foundation resources, and clinical guidelines.

Active Clinical Trials for Adrenocortical Carcinoma

Use this Adrenocortical Carcinoma clinical trial finder to see the 19 studies recruiting patients and 3 opening soon in the United States and worldwide, with eligibility criteria in plain English. These studies play a critical role in advancing care for rare cancers conditions and may offer access to treatments not yet widely available. Each trial below is sourced directly from ClinicalTrials.gov, with eligibility criteria translated into plain English to help patients and caregivers evaluate whether a study may be a fit.

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Note: Trial recruitment statuses on ClinicalTrials.gov may not immediately reflect recent FDA decisions, sponsor announcements, or enrollment changes. Always confirm a trial's current status directly with the study coordinator before making plans.

27 active trials worldwide
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RECRUITINGPHASE1Recently updatedNCT06487481

Study of Preoperative Radiation Therapy in Participants With Resectable Recurrent Abdominal Adrenocortical Carcinoma

Intervention: Surgical resection, Preoperative RT

Sponsor: National Cancer Institute (NCI)

Background:

Adrenocortical carcinoma (ACC) is a rare cancer of the adrenal glands. ACC often returns after tumors are removed with surgery. Less than 35% of people with ACC survive 5 years after diagnosis.

Objective:

Ages 18 Years – 120 Years1 location
Started Oct 2026Updated todayEst. Dec 2030 (~4y 2m)
NOT YET RECRUITINGPHASE1Recently updatedNCT07715903

Hepatic Artery Infusion of Carfilzomib in Participants With Liver Metastatic Disease Previously Treated With Hepatic Artery Infusion Pump Therapy

Intervention: Carfilzomib

Sponsor: National Cancer Institute (NCI)

Background:

Ages 18 Years – 120 Years1 location
Started Oct 2026Updated todayEst. Dec 2029 (~3y 3m)
RECRUITINGRecently updatedNCT05237934

Tissue Procurement and Natural History Study of Neuroendocrine Neoplasms (NENs) Including Adrenocortical Carcinoma (ACC)

Sponsor: National Cancer Institute (NCI)

Background:

Ages 18 Years – 120 Years1 location
Started Apr 2022Updated 5 days agoEst. Dec 2032 (~6y 3m)
NOT YET RECRUITINGPHASE2Recently updatedNCT07743138

STOMP OUT: A Phase 2 Study To Evaluate The Effects Of Ivonescimab In Patients With Unresectable/Metastatic Adrenocortical Carcinoma (ACC) Or Unresectable Pheochromocytoma/Paraganglioma (PPGL)

Intervention: Ivonescimab

Sponsor: M.D. Anderson Cancer Center · Summit Therapeutics

To learn if ivonescimab can help to control previously treated, locally advanced or metastatic ACC or PPGL.

Ages 18 Years+1 location
Started Feb 2027Updated 1 week agoEst. May 2028 (~1y 7m)
RECRUITINGPHASE2Recently updatedNCT06900595

Testing the Addition of an Anti-Cancer Drug, Cabozantinib to the Immunotherapy Drug Cemiplimab (REGN2810), in Adolescents and Adults With Advanced Adrenocortical Cancer

Intervention: Biospecimen Collection, Cabozantinib, Cemiplimab, Computed Tomography, Magnetic Resonance Imaging

Sponsor: National Cancer Institute (NCI)

This phase II trial compares the effect of giving cabozantinib with or without cemiplimab in patients with adrenocortical cancer that has spread to nearby tissue or lymph nodes (locally advanced), and that cannot be removed by surgery (unresectable) or that has come back after a ...

Ages 12 Years+81 locations
Started Feb 2026Updated 1 week agoEst. Jun 2029 (~2y 8m)
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Active trial locations71 cities in the US
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Trial Pipeline

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Data from ClinicalTrials.gov, U.S. National Library of Medicine.
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Related Rare Cancers Conditions

Other rare diseases in the rare cancers category. Patients with Adrenocortical Carcinoma may find relevant research, shared treatment pathways, or overlapping clinical trials among these related conditions.

Companies Developing Adrenocortical Carcinoma Treatments

9 pharmaceutical companies have Adrenocortical Carcinoma in their rare disease portfolio

Frequently Asked Questions About Adrenocortical Carcinoma