Cancer More Cancer Types How Adrenocortical Carcinoma Is Treated By Steph Coelho Steph Coelho Twitter Steph Coelho is a freelance health writer, web producer, and editor based in Montreal. She specializes in covering general wellness and chronic illness. Learn about our editorial process Published on September 23, 2021 Medically reviewed Verywell Health articles are reviewed by board-certified physicians and healthcare professionals. These medical reviewers confirm the content is thorough and accurate, reflecting the latest evidence-based research. Content is reviewed before publication and upon substantial updates. Learn more. by Doru Paul, MD Medically reviewed by Doru Paul, MD Doru Paul, MD, is triple board-certified in medical oncology, hematology, and internal medicine. He is an associate professor of clinical medicine at Weill Cornell Medical College and attending physician in the Department of Hematology and Oncology at the New York Presbyterian Weill Cornell Medical Center. Learn about our Medical Expert Board Print Table of Contents View All Table of Contents Surgeries and Procedures Biologics and Targeted OTC and Alternative Adrenocortical carcinoma is cancer of the adrenal cortex. The adrenal glands are hormone-secreting glands located on top of the kidneys. Their outer layer is called the adrenal cortex. Doctors often diagnose this rare form of cancer in its later stages, after it has spread (metastasized), because initial symptoms are nonexistent, vague, or hard to spot. First-line treatment for adrenocortical carcinoma may include surgery, radiation therapy, or chemotherapy. Doctors may use a combination of these therapies to treat this type of cancer. Additionally, new treatments are emerging for cancer of the adrenal cortex, including biologics and targeted therapy. FatCamera / Getty Images Surgeries and Specialist-Driven Procedures The standard treatment for adrenocortical carcinoma typically involves a combination of surgery, chemotherapy, and radiation therapy. Surgery Doctors may decide to remove the adrenal gland in a procedure called adrenalectomy. Surgery is often the first treatment option used when cancer of the adrenal cortex is caught early and is still considered stage 1 or stage 2. In these stages, the tumor has not yet spread. The surgery usually involves removing the entire affected adrenal gland. In people with this type of cancer, rarely are both adrenal glands affected. Therefore, removing one gland shouldn’t cause side effects since the other adrenal gland will continue to function. If the surgeon notices that nearby lymph nodes are enlarged, the doctor may remove them and send them to pathology to check for cancer cells. Lymph nodes are small structures of the lymphatic system, along which travel fluids and immune cells. When the cancer is in the later stages, after it has spread, surgery will involve also removing nearby affected lymph nodes and tissues. When the cancer has spread to other parts of the body, doctors will use surgery combined with radiation and chemotherapy to provide symptom relief. Surgery alone can’t treat stage 4 adrenocortical carcinoma. Depending on the spread of the cancer, surgery may be laparoscopic (minimally invasive procedure using small incisions to insert a camera and instruments) or involve an abdominal or back incision. Radiation Therapy This type of treatment involves high-energy radiation that kills cancer cells. Doctors may use this treatment combined with surgery and chemotherapy as a palliative care measure to help provide symptom relief and improve a person’s quality of life. While there are two types of radiation therapy, doctors use external radiation therapy to treat adrenocortical carcinoma. This type of therapy involves a machine that targets radiation at the cancer. Radiation therapy lasts for several weeks and involves multiple sessions throughout the week. You shouldn’t experience any pain during radiation treatment itself. However, side effects may include: NauseaVomitingDiarrhea Hair loss in the area receiving radiation therapySkin changes in the area receiving radiation therapyFatigue Doctors might use radiation therapy to treat early-stage 1 or 2 adrenocortical carcinoma if the surgery didn’t remove all the cancer. Treatment after surgery is called adjuvant therapy. It aims to kill any remaining cancer cells that may have been left behind and are too small to be visibly detected. Adjuvant therapy minimizes the risk of cancer recurring. Radiation Therapy for Cancer Chemotherapy Chemotherapy drugs prevent cancer cells from growing, or it kills them outright. Chemotherapy is often given intravenously, while some chemotherapy drugs can be taken by mouth. In some cases, chemotherapy is delivered directly into the body. This is called regional chemotherapy. With combination chemotherapy, you receive several cancer-killing drugs at once. Chemotherapy administration and drug type depend on what cancer you have and the stage of the cancer. Doctors may use chemotherapy in combination with radiation therapy and surgery to relieve symptoms of adrenocortical carcinoma and improve a person’s quality of life. In people with stages 1 or 2 adrenocortical carcinoma, doctors may also recommend chemotherapy after surgery if the cancer is not entirely removed or there’s a risk of cancer returning. Doctors most commonly prescribe a chemotherapy drug called Lysodren (mitotane) to treat adrenal gland cancers. It’s taken in pill form multiple times a day. Because Lysodren blocks hormone production in noncancerous adrenal gland tissue, it can cause side effects such as: FatigueWeakness Vomiting DiarrheaNauseaConfusionRashes You may need to take hormone pills to make up for reduced hormone production. However, in some people, Lysodren can actually help reverse the effects of hormone overproduction. Chemotherapy may also cause the following side effects even if hormone production isn’t affected: NauseaVomiting Hair lossAppetite lossRashesSores in the mouthDiarrhea Weakened immune systemAnemiaFatigueShortness of breath Chemotherapy Side Effects: What to Expect Doctors may also prescribe other chemotherapy drugs in combination with mitotane, such as: Platinol (cisplatin)Adriamycin (doxorubicin)VP-16 (etoposide)Zanosar (streptozocin)Taxol (paclitaxel)Carac (5-fluorouracil)Oncovin (vincristine) An ongoing clinical trial is studying the effects of Cabometyx (cabozantinib-s-malate), a chemotherapy drug. The researchers hope to find an effective option for treating late-stage adrenocortical carcinoma that produces fewer side effects than Lysodren. The trial has an estimated completion date of June 2022. Biologics and Targeted Therapies These are new therapies currently being studied in clinical trials. They show promise for the treatment of rare cancers, including adrenocortical carcinoma. Biologic therapy uses drugs derived from living organisms. Immunotherapy is one type of biologic therapy. It uses the body’s immune system to combat cancer. The results of a small clinical trial involving 16 people with adrenal cortex cancer found that immunotherapy with pembrolizumab was moderately effective in preventing the progression of the cancer. It also produced minimal adverse effects. Targeted therapy involves drugs that target and attack specific cancer cells. Unlike traditional treatments like chemotherapy and radiation, targeted therapy doesn’t damage noncancerous cells. Multiple trials looking into the efficacy and tolerability of targeted therapy drugs are currently ongoing. Over-the-Counter (OTC) and Alternative Therapies There is no OTC treatment or home remedy for cancer. It’s your choice if you wish to take supplements or try alternative therapies. However, you should always talk to your doctor about taking supplements or herbal remedies. Additionally, while complementary and alternative therapies (CAM) shouldn’t replace traditional treatments for cancer, you may find them helpful for managing cancer symptoms or cancer treatment side effects. Summary Adrenocortical carcinoma is a rare type of cancer of the adrenal glands. If found in early stages, it is treated with surgery. Follow-up treatment may be with radiation therapy, chemotherapy, or biologic therapy. For later-stage cancer, these treatments may be used as well. A Word From Verywell Despite being a rare cancer, treatments are available for adrenocortical carcinoma. After receiving a diagnosis of adrenocortical carcinoma, you may feel overwhelmed and wonder about the treatment options available. Treatment will depend primarily on the stage of the cancer. Talk to your oncologist about treatment options. Don’t be afraid to ask your doctor about potential side effects and the best ways to manage them. Was this page helpful? Thanks for your feedback! Limiting processed foods and red meats can help ward off cancer risk. These recipes focus on antioxidant-rich foods to better protect you and your loved ones. Sign up and get your guide! Sign Up You're in! Thank you, {{form.email}}, for signing up. There was an error. Please try again. What are your concerns? Other Inaccurate Hard to Understand Submit 11 Sources Verywell Health uses only high-quality sources, including peer-reviewed studies, to support the facts within our articles. Read our editorial process to learn more about how we fact-check and keep our content accurate, reliable, and trustworthy. National Cancer Institute. Adrenocortical carcinoma treatment. National Cancer Institute. Adrenocortical carcinoma treatment (adult) (PDQ®)—patient version. American Cancer Society. Treatment choices by stage of adrenal cancer. American Cancer Society. Surgery for adrenal cancer. American Cancer Society. Radiation therapy for adrenal cancer. American Cancer Society. Chemotherapy for adrenal cancer. American Cancer Society. Chemotherapy side effects. U.S. National Library of Medicine. Cabozantinib in advanced adrenocortical carcinoma (CaboACC). Habra, MA, et al. Phase II clinical trial of pembrolizumab efficacy and safety in advanced adrenocortical carcinoma. Journal for Immunotherapy of Cancer. 2019;7:253. doi:10.1186/s40425-019-0722-x Alyateem, G, et al. Current status and future of targeted therapy in adrenocortical cancer. Front. Endocrinol. 2021. doi: 10.3389/fendo.2021.613248 American Cancer Society. What are the risks of not using mainstream cancer treatment. Additional Reading Else, T, et al. Adrenocortical carcinoma. Endocr Rev. 2013;35(2): 282-326. doi: 10.1210/er.2013-1029