Adrenocortical Carcinoma: A Plain-Language Guide

This page is for people who have been told they have adrenocortical carcinoma, and their families. It covers what it is, how it is found, and its signs. It also explains how treatment is planned, and when to get help.

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
• How much experience the care team has

Testing may include hormone tests and scans. Doctors who know adrenal tumors may also review your case. Staging is how doctors describe how far the cancer has grown. It looks at tumor size, nearby tissue, lymph nodes, and spread to far parts of the body.

Ask for a copy of the pathology report and the scan summary. Was the diagnosis a surprise, or is the wording unclear? Then ask if a pathologist who knows this cancer has looked at the tissue. A pathologist is a doctor who studies tissue under a microscope.

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 (a stress hormone): weight gain in the face, facial hair growth, weak muscles, and high blood sugar or blood pressure.
• Too much aldosterone (a hormone that controls salt and water): high blood pressure, weak muscles or cramps, peeing often, and strong thirst.
• Extra sex hormones: new facial or body hair, acne, a deeper voice, missed periods, unexpected bleeding after menopause, or breast growth in men.

These signs have many causes besides cancer. Still, check with your doctor if you have them.

Some of these tumors make extra hormones. So both cancer doctors and hormone doctors may help with your symptoms and your plan.

That is why a broad label is only the start. Many things can change the plan. These include the subtype, grade (how fast the cells look like they grow), and stage. Gene findings in the tumor, your symptoms, and your overall health matter too.

Surgery to take out the adrenal gland is a main option when the tumor can be taken out. Other care may include mitotane (a drug for this cancer), chemo, radiation, care for symptoms, or a clinical trial. Which of these come up depends on the stage. It also depends on whether the cancer has come back.

This list names kinds of care. It is not advice. The team that knows your full diagnosis can explain which choices fit, and why.

You can ask how often the center treats this cancer. You can ask if a tumor board will review your case. A tumor board is a group of doctors from different fields who plan care together. You can also ask if a second look at the tissue would help. A second opinion can confirm a plan. It can also suggest other choices.

• The pathology report, and if possible, where the slides are stored
• Imaging reports, and copies of the images
• Current medicines, symptoms, and major health problems
• A written list of test results you are still waiting on
• Questions about specialists, treatment goals, and clinical trials

You do not need to learn every medical detail at once. The goal is to leave knowing three things:

• what is confirmed
• what is still pending
• who owns the next step

Hormone changes from this cancer, and from its treatment, can affect blood pressure, blood sugar, and body salts. Some of those changes need care quickly.

Mitotane matters here. It lowers the hormones the adrenal gland makes. Then the body may not cope well with physical stress. Tell your doctor right away about any serious infection, illness, or injury. Also tell them before any surgery, even 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. Do not drive yourself.
• 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 right away 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 right away 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. On mitotane, also call right away for extreme tiredness, confusion, nausea, diarrhea, belly pain, or salt cravings. For emergencies call 911 or go to the ER.

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. Ask what you should do about each one.

• National Cancer Institute — Symptoms of Adrenocortical Carcinoma
• National Cancer Institute — Treatment for Adrenocortical Carcinoma
• MedlinePlus — Mitotane
• CDC — Watch Out for Fever (Preventing Infections in Cancer Patients)