Short answer · Medically reviewed summary · Last updated: 2026-05-08
The primary treatment for Adrenal Cortical Carcinoma (ACC) is complete surgical resection, often followed by adjuvant therapy with mitotane to reduce the risk of recurrence. Because Adrenal Cortical Carcinoma (ACC) is a rare and aggressive malignancy, treatment must be personalized by a multidisciplinary oncology team based on the tumor's stage, grade, and molecular profile. What are the primary treatments for Adrenal Cortical Carcinoma (ACC)? Surgery remains the gold standard for localized Adrenal Cortical Carcinoma (ACC).
5 people with Adrenal Cortical Carcinoma (ACC) have shared their first-person experience on this question at DiseaseMaps.
What are the best treatments for Adrenal Cortical Carcinoma (ACC)?
Treatments for Adrenal Cortical Carcinoma (ACC): what real patients say works for them, alongside a medically reviewed overview citing sources like NIH GARD and Orphanet.
The primary treatment for Adrenal Cortical Carcinoma (ACC) is complete surgical resection, often followed by adjuvant therapy with mitotane to reduce the risk of recurrence. Because Adrenal Cortical Carcinoma (ACC) is a rare and aggressive malignancy, treatment must be personalized by a multidisciplinary oncology team based on the tumor's stage, grade, and molecular profile.
What are the primary treatments for Adrenal Cortical Carcinoma (ACC)?
Surgery remains the gold standard for localized Adrenal Cortical Carcinoma (ACC). When a complete (R0) resection is achieved, the prognosis is significantly improved. Following surgery, clinicians often prescribe mitotane (Lysodren), an adrenolytic medication that specifically targets adrenal cells. In cases of advanced or metastatic Adrenal Cortical Carcinoma (ACC), systemic chemotherapy—most commonly the combination of etoposide, doxorubicin, and cisplatin (EDP) alongside mitotane—is the standard of care.
Which specialists should be on my care team?
Managing Adrenal Cortical Carcinoma (ACC) requires a specialized, high-volume center because of the disease's complexity. Your care team should ideally include:
- Endocrine Surgeons: Experienced in high-volume adrenal surgery.
- Medical Oncologists: Specialized in rare endocrine malignancies.
- Endocrinologists: To manage hormonal imbalances caused by hormone-secreting tumors.
- Radiation Oncologists: If local control is needed for specific metastatic sites.
- Genetic Counselors: To evaluate for hereditary syndromes like Li-Fraumeni syndrome.
Are there emerging treatments for Adrenal Cortical Carcinoma (ACC)?
Research is actively exploring targeted therapies and immunotherapies, particularly for patients who do not respond to traditional chemotherapy. Clinical trials are currently investigating the use of immune checkpoint inhibitors and molecularly targeted agents. Participation in clinical trials is highly encouraged for patients with recurrent or refractory Adrenal Cortical Carcinoma (ACC) to access novel therapeutic approaches.
Next steps
- Consult with an adrenal oncology specialist at a major academic medical center.
- Connect with the 265 members of the DiseaseMaps.org community to share experiences.
- Discuss genetic testing with a counselor to identify potential underlying hereditary factors.
- Search ClinicalTrials.gov for active trials specifically investigating Adrenal Cortical Carcinoma (ACC).
Medical disclaimer: This information is for educational purposes only and does not constitute medical advice; always consult your personal physician for diagnosis and treatment decisions.
References
- NIH Genetic and Rare Diseases (GARD) Information Center: Adrenal Cortical Carcinoma
- Orphanet: Adrenocortical carcinoma (ORPHA:364)
- American Association of Endocrine Surgeons (AAES) Guidelines
- National Cancer Institute (NCI) PDQ: Adrenocortical Carcinoma Treatment
Posted Jun 11, 2017 by Grace 500
For stage 4 patients where the cancer has metastasized to other organs, the treatment of choice is a combination of the drug Mitotane plus intravenous chemotherapy consisting of etoposide, doxorubicin (also called adriamycin) and cisplatin. The goal is usually to shrink the cancer enough to make surgery possible.
For those where the cancer has spread on the surfaces of abdominal organs, a procedure that combines both abdominal surgery and a procedure called HIPEC (heated intraperitoneal chemotherapy). HIPEC is a warmed and concentrated solution of cisplatin that is used to bathe the entire abdomen for approximately 90 to 120 minutes following surgical removal of all visible cancer. The drug comes into direct contact with microscopic remnants of cancer cells and kills them. The abdomen is then flushed to remove the chemotherapeutic agent, and a drug is given intravenously to prevent systemic effects and preserve kidney function.
Surgery is currently the only known cure for ACC. There are other techniques that are effective and include: radio frequency ablation, chemo-emobolization and low dose or stereotactic radiation. The first two are generally used to treat liver metastases. Low dose radiation is sometimes warranted when surgical margins are not clean; radiation is used to treat the surgical bed with variable results concerning recurrence. Stereotactic radiation can be used on metastases generally 0.5 to 2.0 cm and is most often used to treat metastases in the lungs.
Posted Jun 11, 2017 by Sherry 200
Posted Mar 9, 2018 by Accmom 2500
Posted May 22, 2018 by Kim 1600
Chimiothérapie cisplatine et etoposide
Chirurgie de la tumeur
Posted Feb 26, 2019 by Flor 400
