Adrenocortical Carcinoma Diagnosis: Questions to Ask

Adrenocortical carcinoma forms in the outer layer of an adrenal gland, called the adrenal cortex. The tests you are asked to do are answering two separate questions. Is there a tumor? And is that tumor releasing extra hormones? That is why your list mixes urine collections and blood draws with scans.

Some adrenal cortex tumors make extra cortisol, aldosterone, testosterone, or estrogen. NCI links each hormone to specific signs.

Too much cortisol can cause weight gain in the face, neck, and trunk with thin arms and legs, muscle weakness, high blood sugar, and high blood pressure. Too much aldosterone can cause high blood pressure, muscle weakness or cramps, frequent urination, and feeling thirsty. Too much testosterone can cause facial hair, acne, balding, a deeper voice, or no menstrual periods in women. Too much estrogen can cause irregular periods or vaginal bleeding after menopause in women. In men it can cause breast growth, lower sex drive, or impotence.

Tumors that do not make extra hormones may instead cause a lump in the abdomen, pain in the abdomen or back, or a feeling of fullness.

• Twenty-four-hour urine test. Urine is collected for a full day to measure cortisol or 17-ketosteroids. Higher than normal amounts can be a sign of disease in the adrenal cortex.
• Low-dose dexamethasone suppression test. You take small doses of dexamethasone. Then cortisol is measured from blood, or from urine collected over three days. It checks whether your adrenal gland is making too much cortisol.
• High-dose dexamethasone suppression test. Same idea with higher doses. This one separates two causes. The cortisol may be coming from the adrenal gland itself. Or it may be driven by signals from the pituitary gland, a small gland at the base of the brain. Ask which of those two your result points to.
• Blood chemistry study. Measures substances such as potassium and sodium. An unusual amount can be a sign of disease.
• Blood hormone studies. A blood sample checked for the amounts of certain hormones released by organs.

CT and MRI of the abdomen make detailed pictures. CT may use injected dye to show structures more clearly. A PET scan uses radioactive glucose. Malignant tumor cells show up brighter, because they are more active and take up more glucose. An MIBG scan is used to tell adrenocortical carcinoma apart from pheochromocytoma, a different adrenal tumor.

Adrenal angiography and adrenal venography put dye into the adrenal arteries or veins to look at blood flow and blockages. During venography, a thin tube called a catheter may be threaded into the vein. It can draw a blood sample to check for abnormal hormone levels.

A biopsy removes cells or tissue so a pathologist can look at them under a microscope for signs of cancer. It may be done as a fine-needle aspiration or a core biopsy.

• Stage I: the tumor is 5 centimeters or smaller and is found in the adrenal gland only.
• Stage II: the tumor is larger than 5 centimeters and is found in the adrenal gland only.
• Stage III: the tumor can be any size and has spread to nearby lymph nodes, to nearby tissues or organs such as the kidney, diaphragm, pancreas, spleen, or liver, or to major blood vessels such as the renal vein or vena cava.
• Stage IV: the tumor is any size, may have spread to nearby lymph nodes, and has spread to other parts of the body such as the lung, bone, or peritoneum.

Prognosis and treatment options depend on several things. They depend on the stage. They depend on whether the tumor can be completely removed by surgery. They depend on whether the cancer has been treated before, and on your general health. NCI states that adrenocortical carcinoma may be cured if treated at an early stage.

• Is my tumor making extra hormones, and which ones? What did the 24-hour urine and blood hormone results show?
• If a dexamethasone suppression test was done, did it point to the adrenal gland or the pituitary gland?
• Which scans have been done, and what is still needed to describe the extent of disease?
• Was an MIBG scan considered to rule out pheochromocytoma?
• What stage is this, and which specific finding set that stage?
• Can the tumor be completely removed by surgery?
• Which pending result would change the plan, and who will call me with it?

Ask for the exact diagnosis in writing, including subtype and grade when those apply. Before you leave, get one sentence you can repeat: what is confirmed, what is still pending, and what decision comes next.