Overview
Adrenocortical carcinoma (ACC), also known as adrenal cortical carcinoma or adrenal cortex cancer, is a rare and aggressive malignant tumor arising from the cortex (outer layer) of the adrenal glands. The adrenal glands are small organs located on top of each kidney that produce essential hormones including cortisol, aldosterone, and androgens. ACC can occur at any age but shows a bimodal distribution with peaks in childhood and in the fourth to fifth decades of life. Approximately 50-60% of ACCs are hormonally functional, meaning they produce excess hormones that cause distinct clinical syndromes. When the tumor is hormone-secreting, patients may present with Cushing syndrome (due to excess cortisol, causing weight gain, moon face, skin fragility, hypertension, and diabetes), virilization (excess androgens causing hirsutism, deepening of the voice, and acne in women), feminization (excess estrogen in males), or hyperaldosteronism (causing hypertension and low potassium levels). Non-functioning tumors may remain clinically silent until they grow large enough to cause abdominal pain, a palpable mass, or symptoms from local compression of adjacent structures. Many cases are now discovered incidentally during imaging performed for other reasons (adrenal incidentalomas). Treatment of ACC typically involves complete surgical resection (adrenalectomy), which remains the cornerstone of therapy and offers the best chance of cure when the tumor is localized. Mitotane, an adrenolytic drug, is used as adjuvant therapy after surgery and in advanced or metastatic disease to suppress adrenal hormone production and inhibit tumor growth. Cytotoxic chemotherapy regimens, most commonly etoposide, doxorubicin, and cisplatin combined with mitotane (EDP-M), are used for advanced disease. Radiation therapy may be considered in select cases. Despite treatment, ACC carries a poor overall prognosis, particularly in advanced stages, with five-year survival rates varying widely depending on stage at diagnosis. Genetic predisposition syndromes such as Li-Fraumeni syndrome (TP53 mutations), Beckwith-Wiedemann syndrome, and multiple endocrine neoplasia type 1 (MEN1) are associated with increased risk of ACC.
Also known as:
Clinical phenotype terms— hover any for plain English:
Variable
Can be inherited in different ways depending on the underlying gene
Variable
Can begin at different ages, from infancy through adulthood
FDA & Trial Timeline
10 eventsM.D. Anderson Cancer Center — PHASE2
Wake Forest University Health Sciences — PHASE1
National Cancer Institute (NCI) — PHASE1
National Cancer Institute (NCI) — PHASE2
Kernel
Muhammet Talha Ceran, MD
Daiichi Sankyo — PHASE1
Azienda Socio Sanitaria Territoriale degli Spedali Civili di Brescia — PHASE2
Sun Yat-sen University — NA
University Hospital, Strasbourg, France
Data sourced from FDA regulatory filings and ClinicalTrials.gov. Updated periodically.
Treatments
1 availableTamoxifen Citrate
In women with DCIS, following breast surgery and radiation, tamoxifen citrate tablets are indicated to reduce the risk of invasive breast cancer.
Rare Disease Specialist
Rare Disease Specialist
Rare Disease Specialist
Treatment Centers
8 centersBaylor College of Medicine Rare Disease Center ↗
Baylor College of Medicine
📍 Houston, TX
🏥 NORDStanford Medicine Rare Disease Center ↗
Stanford Medicine
📍 Stanford, CA
🔬 UDNNIH Clinical Center Undiagnosed Diseases Program ↗
National Institutes of Health
📍 Bethesda, MD
🔬 UDNUCLA UDN Clinical Site ↗
UCLA Health
📍 Los Angeles, CA
🔬 UDNBaylor College of Medicine UDN Clinical Site ↗
Baylor College of Medicine
📍 Houston, TX
🔬 UDNHarvard/MGH UDN Clinical Site ↗
Massachusetts General Hospital
📍 Boston, MA
🏥 NORDMayo Clinic Center for Individualized Medicine ↗
Mayo Clinic
📍 Rochester, MN
👤 Mayo Clinic Center for Individualized Medicine
🏥 NORDUCLA Rare Disease Day Program ↗
UCLA Health
📍 Los Angeles, CA
Financial Resources
1 resourcesTravel Grants
No travel grants are currently matched to Adrenocortical carcinoma.
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Common questions about Adrenocortical carcinoma
What is Adrenocortical carcinoma?
Adrenocortical carcinoma (ACC), also known as adrenal cortical carcinoma or adrenal cortex cancer, is a rare and aggressive malignant tumor arising from the cortex (outer layer) of the adrenal glands. The adrenal glands are small organs located on top of each kidney that produce essential hormones including cortisol, aldosterone, and androgens. ACC can occur at any age but shows a bimodal distribution with peaks in childhood and in the fourth to fifth decades of life. Approximately 50-60% of ACCs are hormonally functional, meaning they produce excess hormones that cause distinct clinical syndr
Are there clinical trials for Adrenocortical carcinoma?
Yes — 17 recruiting clinical trials are currently listed for Adrenocortical carcinoma on UniteRare. See the clinical trials section on this page for phase, sponsor, and site details sourced from ClinicalTrials.gov.
Which specialists treat Adrenocortical carcinoma?
25 specialists and care centers treating Adrenocortical carcinoma are listed on UniteRare, sourced from ClinicalTrials.gov principal investigators, published research, and the NPPES NPI registry.