Immunotherapy for Adrenal Cancer

Immunotherapy is the use of medicines to help a person’s own immune system recognize and destroy cancer cells. These drugs have different side effects from those of chemotherapy.

Immunotherapy is used to treat some adrenocortical carcinomas (ACC) and neuroblastomas. For more information on the use of immunotherapy in neuroblastoma, see Immunotherapy for Neuroblastoma.

Immune checkpoint inhibitors for adrenocortical carcinoma

Just as it’s important that immune cells are activated to fight infections and cancer, it’s also important that they turn off when they are no longer needed.

Checkpoint proteins on immune cells act as brakes to slow down immune system responses. Cancer cells sometimes use these checkpoints to keep from being attacked by the immune system.

Drugs that target these checkpoint proteins are called immune checkpoint inhibitors. They can help the immune system fight off cancer cells.

PD-1 is a checkpoint protein on certain immune cells called T cells.

Pembrolizumab (Keytruda) is a drug that targets the PD-1 protein. This drug might be an option to treat ACC that has spread or can’t be removed with surgery. By blocking PD-1, this drug boosts the immune response against cancer cells. This can often shrink tumors or slow their growth.

This drug is given into a vein (IV) as an infusion. It is typically given every 3 or 6 weeks. It can be used with or without mitotane.

Nivolumab (Opdivo, Opdivo Qvantig) is a PD-1 inhibitor that has been used in combination with ipilimumab, another type of immune checkpoint inhibitor, to treat advanced ACC.

CTLA-4 is a checkpoint protein on some T cells. Ipilimumab (Yervoy) is a CTLA-4 inhibitor that might be used in combination with nivolumab for advanced ACC.

Possible side effects of immune checkpoint inhibitors

Side effects of these drugs can include fatigue, cough, skin rash, poor appetite, constipation, diarrhea, and joint pain.

Other, more serious side effects occur less often.

Infusion reactions

Infusion reactions are like allergic reactions and can include:

  • Fever, chills, flushing of the face
  • Rash, itchy skin
  • Feeling dizzy
  • Wheezing and trouble breathing

It’s important to tell your doctor or nurse right away if you have any of these symptoms while getting these drugs.

Autoimmune reactions

These drugs can also cause immune cells to attack normal healthy cells throughout your body. This can lead to problems with your lungs, gut, and hormone-making glands like the thyroid, kidneys, or other organs.

It’s very important to report any new side effects to your healthcare team right away. If serious side effects do occur, treatment might need to be stopped. You might need other treatment to stop these side effects.

To learn more about the side effects of immune checkpoint inhibitors, see Immune Checkpoint Inhibitors and Their Side Effects.

More information about immunotherapy

To learn more about how drugs that work on the immune system are used to treat cancer, see Cancer Immunotherapy.

To learn about some of the side effects listed here and how to manage them, see Managing Cancer-related Side Effects.

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Developed by the 黑料大湿Posts Cancer Society medical and editorial content team with medical review and contribution by the 黑料大湿Posts Society of Clinical Oncology (ASCO).

Habra MA, Stephen B, Campbell M, Hess K, Tapia C, Xu M, Rodon Ahnert J, et al. Phase II clinical trial of pembrolizumab efficacy and safety in advanced adrenocortical carcinoma. J Immunother Cancer. 2019 Sep 18;7(1):253.

Klein O, Senko C, Carlino MS, et al. Combination immunotherapy with ipilimumab and nivolumab in patients with advanced adrenocortical carcinoma: a subgroup analysis of CA209-538. Oncoimmunology. 2021;10(1):1908771. Published 2021 Apr 12.

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.

Raj N, Zheng Y, Kelly V, Katz SS, Chou J, Do RKG, Capanu M, et al.PD-1 Blockade in Advanced Adrenocortical Carcinoma. J Clin Oncol. 2020 Jan 1;38(1):71-80.

Last Revised: October 5, 2026

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