The short answer
Adrenocortical carcinoma is a rare cancer that forms in the outer layer of an adrenal gland. The adrenal glands sit above the kidneys and make hormones. Because it is uncommon, the exact pathology and experience of the treating team can be especially important.
Adrenocortical carcinoma is a rare cancer that forms in the outer layer of an adrenal gland. The adrenal glands sit above the kidneys and make hormones.
Evaluation may include hormone tests, imaging, and review by specialists familiar with adrenal tumors. Staging looks at tumor size, nearby tissues, lymph nodes, and distant spread.
Some tumors make extra hormones, so symptoms and treatment planning can involve both cancer specialists and hormone specialists.
A rare-cancer diagnosis is reasonable to review with a team that knows the condition.
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The full explanation.
The simple version
Adrenocortical carcinoma is a rare cancer. It forms in the outer layer of an adrenal gland. The adrenal glands sit above the kidneys and make hormones.
Rare does not mean unknowable. It does mean a few things deserve close attention. The exact pathology name. The tests used to confirm it. And how much experience the care team has.
How it is found
Workup may include hormone tests, imaging, and review by specialists who know adrenal tumors. Staging looks at tumor size, nearby tissues, lymph nodes, and distant spread.
Ask for a copy of the pathology report and the imaging summary. Was the diagnosis unexpected, or is the wording unclear? Ask whether a pathologist with experience in this condition has looked at the tissue.
What it can feel like
Many people first notice a lump in the abdomen, or pain in the abdomen or back. Some feel full in the belly.
When a tumor makes extra hormones, the signs depend on which hormone. Too much cortisol can cause a round face, fat at the back of the neck, thin arms and legs, muscle weakness, and high blood sugar and blood pressure. Too much aldosterone can cause high blood pressure, muscle weakness or cramps, frequent urination, and heavy thirst. Extra sex hormones can cause new facial or body hair, acne, a deeper voice, missed periods, unexpected bleeding after menopause, or breast growth in men.
These signs have many other causes besides cancer. Still, check with your doctor if you have them.
What makes this condition distinct
Some of these tumors make extra hormones. So symptoms and treatment planning can involve both cancer specialists and hormone specialists.
That is why a broad label is only the start. Subtype, grade, stage, molecular findings, symptoms, and overall health may all change the conversation.
How treatment is planned
Surgery to remove the adrenal gland is a main option when the tumor can be removed. Other care may include mitotane, chemotherapy, radiation, symptom treatment, or a clinical trial. Which of these come up depends on stage and on whether the cancer has returned.
This list describes categories of care, not a recommendation. The team that knows the full diagnosis can explain which choices fit, and why.
Building the right team
You can ask how often the center treats this condition. You can ask whether a multidisciplinary tumor board will review it. You can also ask whether a second pathology opinion would add anything useful. A second opinion can confirm a plan as well as suggest alternatives.
What to take to the next visit
- The pathology report, and if possible, where the slides are stored
- Imaging reports, and copies of the images
- Current medicines, symptoms, and major health conditions
- A written list of test results you are still waiting on
- Questions about specialists, treatment goals, and clinical trials
The goal is not to learn every medical detail at once. It is to leave knowing three things. What is confirmed. What is still pending. And who owns the next step.
When to get help sooner
Hormone changes from this cancer, and from its treatment, can affect blood pressure, blood sugar, and body salts. Some of those changes need attention quickly.
Mitotane matters here. It lowers the hormones the adrenal gland makes, so the body may not be able to handle physical stress. Tell your doctor right away about any serious infection, illness, or injury, and before any surgery, including dental surgery.
- Call 911 or go to an emergency department if you become extremely weak, lightheaded, or hard to wake, or if you have severe belly or back pain that comes on suddenly, chest pain, trouble breathing, or a seizure.
- Call your care team immediately, day or night, if you have a temperature of 100.4°F (38°C) or higher while on chemotherapy or on mitotane. CDC calls fever during chemotherapy a medical emergency, and an infection can also tip someone whose adrenal hormones are suppressed into a crisis. If you cannot reach your team quickly, go to an emergency department and say straight away that you are having cancer treatment.
- Call your care team the same day if you feel faint or dizzy when you stand, you are vomiting and cannot keep fluids down, or your blood pressure readings are much higher than usual.
- Call your care team within a day or two if you notice new muscle weakness or cramps, much more thirst or urination than normal, blood sugar readings that are climbing, or new swelling or weight change.
Do not change or stop any of your medicines on your own. Ask your team which symptoms come from the hormones and which come from the treatment, and what each one should prompt you to do.
Sources
Words to know
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Common questions
What is adrenocortical carcinoma?
Adrenocortical carcinoma is a rare cancer that forms in the outer layer of an adrenal gland. The adrenal glands sit above the kidneys and make hormones.
How is it diagnosed?
Evaluation may include hormone tests, imaging, and review by specialists familiar with adrenal tumors. Staging looks at tumor size, nearby tissues, lymph nodes, and distant spread.
How is treatment planned?
Surgery to remove the adrenal gland is a main option when the tumor can be removed. Other care may include mitotane, chemotherapy, radiation, symptom treatment, or a clinical trial depending on stage and recurrence.
Why might a second opinion help?
Rare cancers can have specialized pathology and treatment questions. A second review can confirm the diagnosis and clarify options without committing you to change care.
Questions to ask your doctor
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Your next step
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Sources last checked: 2026-08-13 what this meansLast updated: 2026-08-18Next planned review: 2027-07-22
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How this page was created
Cancer Explained does not originate medical claims. Every page restates guidance already published by the National Cancer Institute, the CDC, the USPSTF and the FDA, in plain language, with the source cited so you can check the original yourself. AI does the translating and organizing; automated checks test claims, citations, clarity and safety before anything publishes. We do not employ clinicians and do not intend to — our work is translation and navigation, not clinical judgment. Nothing here is personal medical advice, and no page can account for your particular situation.
Editorial status: Source checked — This page was written with AI assistance and checked line by line against the sources listed on it. That confirms the sources support what the page says. It is not a medical review, and it does not confirm the page is complete or right for your situation.
Human medical review: not completed. Pages here are not signed off by a clinician before they publish. That is not an oversight we are quietly working around: we restate published guidance and cite it, so the authority belongs to the source rather than to us, and every page names where its claims come from — you can verify us instead of trusting us. Where a volunteer clinician has reviewed a page, their name and credentials appear on it; where no name appears, no clinician has checked it. We are glad to have reviewers and are recruiting them, and we do not hold pages back waiting for one. Use this site to understand your situation and to ask better questions of the people treating you.
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