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.
Source: openFDA drug labeling data. This list may not include all treatments. Always consult your doctor.
Help Paying for Adrenocortical Carcinoma Treatment
Charity funds and drugmaker programs for Adrenocortical Carcinoma, checked at the source. Pick your insurance to see what fits.
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.