Adrenal tumors

Types of adrenal tumors

The most common are nonfunctioning adenomas — benign lesions that are simply monitored. Some tumors produce hormones: an aldosteronoma produces aldosterone, causing persistent high blood pressure and, in some patients, low potassium; a cortisol-producing adenoma produces cortisol, ranging from overt Cushing’s syndrome to mild autonomous excess that worsens blood pressure, blood sugar and bones; a pheochromocytoma produces adrenaline and noradrenaline, causing blood pressure surges.

Less common are adrenocortical carcinoma — a rare, aggressive tumor that often grows large — and metastases from other cancers to the adrenal gland. A substantial share of pheochromocytomas are hereditary, so genetic counseling is recommended for all such patients.

Are there symptoms

Nonfunctioning adenomas cause no symptoms. An aldosteronoma shows up as hard-to-control high blood pressure and, with low potassium, weakness and cramps. Cortisol excess causes weight gain around the abdomen and face, stretch marks, diabetes and osteoporosis. A pheochromocytoma causes episodes of headache, palpitations and sweating with sharp rises in blood pressure.

A large adrenocortical carcinoma may cause flank pain, a feeling of pressure, and rapidly worsening signs of hormone excess.

Examinations needed

• Non-contrast CT — the density of the lesion: a homogeneous lesion with a density of up to 10 Hounsfield units practically confirms a benign adenoma. If density is higher — contrast CT with washout assessment or MRI.
• 1 mg dexamethasone suppression test — to look for cortisol excess; recommended for most patients with an incidentally found lesion.
• Plasma or urine metanephrines — to rule out pheochromocytoma; mandatory before any procedure on a lesion that is not a clear-cut adenoma.
• Aldosterone and renin — with high blood pressure or low potassium; if positive — confirmatory tests and often adrenal vein sampling to determine which side is overproducing.
• If cancer is suspected — sex hormones and their precursors, chest CT.

Adrenal biopsy is not performed when a primary adrenal tumor is suspected: it cannot distinguish an adenoma from cancer and may spread the tumor. It is done only for a suspected metastasis when the result will change treatment, and only after pheochromocytoma has been ruled out.

When surgery is needed

Surgery is recommended for hormonally active tumors: pheochromocytoma, aldosteronoma with one-sided production, overt cortisol excess, and mild cortisol excess when it has consequences. Tumors with features of possible malignancy on CT or MRI, or those that are clearly growing — by more than 20% and at least 5 mm in 6–12 months — are also removed.

Small nonfunctioning adenomas with typical CT features need no surgery and often no further follow-up. A solitary adrenal metastasis is sometimes removed if the primary cancer is under control.

If surgery is not possible now

With aldosterone production from both glands, treatment is with drugs that block the action of aldosterone. For inoperable or metastatic adrenocortical carcinoma, mitotane and chemotherapy are used; individual metastases are sometimes removed. Metastatic pheochromocytoma is treated with radionuclide therapy, chemotherapy or targeted drugs, while keeping blood pressure under control.

How the operation is performed

The standard for benign tumors and most pheochromocytomas is laparoscopic adrenalectomy: the adrenal gland is removed through several small incisions, either through the abdomen or from the back. Before surgery for pheochromocytoma, patients take alpha-blockers for 1–2 weeks and increase fluid and salt intake to avoid sharp swings in blood pressure during removal.

When adrenocortical carcinoma is suspected, especially with signs of invasion, the tumor is usually removed open, en bloc with the surrounding fat and lymph nodes, if necessary with part of the kidney, liver or a vein, without breaching the capsule: the outcome depends most on the completeness of the first operation.

Recovery after surgery

Care follows the enhanced recovery after surgery (ERAS) protocol: early mobilization, early feeding, controlled pain relief. After laparoscopic adrenalectomy, patients are usually discharged within a few days.

After removal of a cortisol-producing tumor, the other adrenal gland is suppressed for a while, so hydrocortisone is given temporarily and tapered once recovery is confirmed by blood tests. After an aldosteronoma, potassium and blood pressure are monitored; after a pheochromocytoma, blood pressure and blood glucose. One healthy adrenal gland is usually enough for a normal life.

Follow-up

After a pheochromocytoma, metanephrines are checked yearly for at least 10 years, and lifelong in hereditary forms, because recurrences and new tumors are possible. After adrenocortical carcinoma, CT is done every 3 months for the first 2 years, then less often; adjuvant mitotane is prescribed at high risk of recurrence. After removal of a benign adenoma, follow-up is usually not needed beyond checking the hormonal effect.

What to bring to the consultation

• CT or MRI discs with reports, including a non-contrast CT with the lesion’s density;
• hormone test results: dexamethasone suppression test, metanephrines, aldosterone and renin, potassium;
• a list of medications, especially for blood pressure;
• records of the underlying cancer, if a metastasis is suspected;
• information about adrenal or endocrine tumors in the family.

If some materials are missing, that is no reason to postpone the consultation: some examinations can be done on site.

The consultation is free of charge. How it goes and how long it takes is described on the page “Oncologist Consultation”.