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Treatment Choices by Stage of Adrenal Cancer
Treatment of adrenal cancer depends on its type, where the cancer started, and how far it has spread. Clinical trials may offer treatment options that are not widely available. Talk to your cancer care team about your treatment plan and goals.
For information about neuroblastoma, see Treating Neuroblastoma.
Adrenocortical carcinoma
Adrenocortical carcinoma (ACC) is the most common type of adrenal cancer. It forms in the outer section (cortex) of the adrenal gland.
Surgery is the main treatment for stage I and stage II adrenal cancers. The entire adrenal gland is removed. Since a person has 2 adrenal glands, removal of the diseased gland does not generally cause problems. If nearby lymph nodes are enlarged, they will be removed as well and checked to see if they have cancer cells.
For many people, no further treatment is necessary. If the tumor had high risk features, immune checkpoint inhibitors or other treatments may be given after surgery to lower the risk that the cancer comes back.
High risk features of adrenal tumors include:
- A high Ki-67 percentage (> 10%). A high Ki-67 score means that tumor cells are dividing quickly.
- The cancer cannot be completely removed.
- The cancer burst open before or during surgery, spilling into the belly.
- The cancer is large and involves blood vessels or the outer surface of the adrenal gland (capsule).
Treatment after surgery to remove all visible cancer is called adjuvant therapy. The goal is to kill any cancer cells that may have been left behind but are too small to be seen. Killing these cells may lower the chance the cancer will come back.
Surgery is the main treatment for stage III adrenocortical carcinoma. The goal of surgery is to remove all the cancer. The adrenal gland with the tumor is always removed. The surgeon might also need to remove tissue around the adrenal gland, including part (or all) of the nearby kidney or part of the liver. The lymph nodes near the adrenal gland will also be removed. After surgery, adjuvant therapy with radiation and/or chemotherapy might be given to help keep the cancer from coming back.
When the cancer cannot be safely removed (unresectable) or has spread to other parts of the body (stage IV), the goal of treatment is to improve symptoms and quality of life.
Observation
This may be an option for people with certain adrenal cancers that are growing relatively slowly and not causing any symptoms. Your doctor may talk about monitoring the cancer with scans with or without blood tests.
Surgery
Some doctors may still recommend surgery. The goal of surgery could be to remove:
- As much of the tumor as possible (debulking).
- An area of spread (metastasis), especially if it is causing symptoms or making hormones.
Hormone therapy
If the tumor is making hormones, treatments to lower the hormone levels or reduce their effects might be tried.
Localized therapy
Ablative therapy or radiation therapy may be used to treat any areas of cancer that are causing symptoms. For example, radiation can help when cancer that has spread to the bones is causing pain.
Systemic therapy
Treatments that can reach throughout the body include chemotherapy, targeted drug therapy, and immunotherapy.
Cancer is called recurrent when it comes back after treatment. Recurrence can be:
- Local (in or near the same place it started)
- Distant (in other organs such as the lungs or bones)
Recurrence may be treated with surgery to remove the cancer or with radiation to destroy the cancer and reduce symptoms. Other treatments such as chemotherapy, targeted drug therapy, or immunotherapy might slow the tumor growth.
Treatment of recurrent adrenocortical carcinoma may provide help temporarily, but many tumors continue to grow over time.
Talk to your care team about your prognosis, treatment options, and goals. It is important that you tell your doctor how you are feeling and what symptoms you are having. They can provide supportive care, whether or not you decide to continue treatment.
Childhood adrenocortical carcinoma
The main treatment for children with adrenocortical carcinoma is surgery with or without chemotherapy.
If adrenocortical carcinoma in a child comes back after initial treatment, targeted drug therapy or immunotherapy may be an option.
Malignant pheochromocytoma
Cancerous pheochromocytomas are those that have spread. About 1 in 10 pheochromocytomas is cancerous.
In these cases, the tumor often cannot be removed completely. Treatments are used to decrease the size of a tumor and slow cancer spread which might improve quality of life and reduce symptoms.
Hormone therapies can be used to lower catecholamine effects and reduce symptoms. Surgery might also be used to lower the hormone levels and symptoms caused by the areas of spread if those areas can be removed.
If localized areas of spread cannot be removed, treatments like ablation, embolization, or radiation might be tried.
If the tumor has spread to multiple areas in the body, local treatments may not be an option. In these cases, if there are no symptoms your care team may watch your tumors closely with imaging tests. If symptoms develop, they may suggest treatments like chemotherapy, targeted drug therapy, or radionuclide therapy.
- Written by
- References
Developed by the American Cancer Society medical and editorial content team with medical review and contribution by the American Society of Clinical Oncology (ASCO).
American Joint Committee on Cancer. Adrenal Cortical. In: AJCC Cancer Staging Manual. 8th ed. New York: Springer. 2018911-9
Berruti A, Fassnacht M, Baudin E, et al. Adjuvant therapy in patients with adrenocortical carcinoma: a position of an international panel. J Clin Oncol. 2010;28(23):e401-e403.
Fassnacht M, Assie G, Baudin E, et al. Adrenocortical carcinomas and malignant phaeochromocytomas: ESMO-EURACAN Clinical Practice Guidelines for diagnosis, treatment and follow-up. Ann Oncol. 2020;31(11):1476-1490.
National Cancer Institute. Adrenocortical Carcinoma Treatment (PDQ). Accessed at https://www.cancer.gov/types/adrenocortical/hp/adrenocortical-treatment-pdq on June 23, 2026.
National Cancer Institute. Childhood Adrenocortical Carcinoma Treatment (PDQ). Accessed at https://www.cancer.gov/types/adrenocortical/hp/child-adrenocortical-treatment-pdq on June 23, 2026.
National Comprehensive Cancer Network. NCCN Clinical Practice Guidelines in Oncology: Neuroendocrine and Adrenal Tumors Version 1.2026 – April 21, 2026. Accessed at https://www.nccn.org/professionals/physician_gls/pdf/neuroendocrine.pdf on June 23, 2026.
Taïeb D, Wanna GB, Ahmad M, et al. Clinical consensus guideline on the management of phaeochromocytoma and paraganglioma in patients harbouring germline SDHD pathogenic variants. Lancet Diabetes Endocrinol. 2023;11(5):345-361.
Taïeb D, Nölting S, Perrier ND, et al. Management of phaeochromocytoma and paraganglioma in patients with germline SDHB pathogenic variants: an international expert Consensus statement. Nat Rev Endocrinol. 2024;20(3):168-184.
Last Revised: October 5, 2026
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