Adrenalectomy: Information & Adrenalectomy Specialists

The adrenal gland consists of the adrenal cortex and the adrenal medulla. Various hormones, known as steroid hormones, are produced in the adrenal cortex. These include

• glucocorticoids (the main one being cortisol),
• mineralocorticoids (the main one being aldosterone), and
• sex hormones.

The adrenal medulla produces the so-called catecholamines (adrenaline, norepinephrine, and dopamine).

Malignant glandular tumors are called carcinomas. Tumors of the adrenal gland may be associated with increased hormone production. This can lead to various clinical presentations that may necessitate an adrenalectomy.

An adrenalectomy may be necessary for two reasons:

• In cases of hormone-producing adrenal tumors
• In cases of malignant adrenal tumors or suspected malignant tumors.

The adrenal gland is pyramid-shaped and sits atop the kidney itself © Peakstock | AdobeStock

Adrenalectomy for Excess Glucocorticoid Production

Excessive production of glucocorticoids leads to Cushing’s syndrome. This condition causes significant changes in affected patients.

Symptoms of Cushing’s syndrome

This results in

• a redistribution of body fat with an accumulation in the trunk (trunk obesity),
• a strikingly round facial shape (moon face), and
• in many cases, an accumulation of fatty tissue in the neck area (buffalo hump).

Another characteristic feature is so-called striae rubrae, which are reddish stretch marks on the skin. They can

• on the torso,
• on the thighs, and
• on the upper arms

.

In women, there is an

• increase in facial hair and
• possibly some chest hair (known as hirsutism).

Menstrual irregularities, including amenorrhea, are also typical of this condition.

The skin becomes noticeably thin and sensitive, and bleeding often occurs.

Other typical symptoms of Cushing’s syndrome include

• high blood pressure and
• disorders of glucose metabolism, including diabetes.

Cushing’s syndrome is a life-threatening condition. If left untreated, it leads to death within 5 years in 50% of patients.

Causes of Cushing’s syndrome

Cushing’s syndrome is most commonly caused by a tumor of the pituitary gland (pituitary tumor). This tumor stimulates the adrenal glands to produce increased amounts of glucocorticoids. The second most common cause is a tumor of the adrenal gland itself, which may be benign or malignant.

In very rare cases, tumors in other organs are the cause, such as small-cell lung cancer. These tumors produce a protein similar to the pituitary-stimulating hormone. This protein can also stimulate the adrenal glands to produce increased amounts of glucocorticoids. This is referred to as a paraneoplastic cause of Cushing’s syndrome.

A pituitary tumor is removed via neurosurgery whenever possible. If this is not possible, an adrenalectomy may be considered as a last resort.

The same applies to the paraneoplastic cause of increased hormone production. The adrenal gland should be removed if it contains a tumor that is causing Cushing’s syndrome.

Adrenalectomy for Excess Mineralocorticoid Production

Increased production of aldosterone leads to what is known as Conn’s syndrome. The overproduction of aldosterone causes less sodium to be excreted by the kidneys. Instead, more potassium is excreted into the urine.

Excess sodium in the body binds water, increasing the volume of fluid in the bloodstream. This leads to high blood pressure, which is often difficult to control. As a result, many patients require three or more different blood pressure medications.

In cases of difficult-to-control high blood pressure combined with low potassium levels, physicians should consider a diagnosis of Conn’s syndrome. However, there are also a number of patients in whom potassium levels are not measured as being too low.

The increased production of aldosterone may be caused by bilateral adrenal hyperplasia. This means that both adrenal glands are equally involved in the increased aldosterone production. In these cases, drug therapy with an aldosterone antagonist is the treatment of choice.

If a unilateral adrenal tumor is causing the excess hormone production, an adrenalectomy should be considered. These tumors are often very small. Therefore, it is sometimes advisable to determine which adrenal gland is responsible for the increased hormone production. This can be determined by measuring hormone levels in the blood.

Adrenalectomy for Excessive Production of Sex Hormones

Excessive production of sex hormones is usually only noticeable when hormones of the opposite sex are produced.

If a woman’s body produces too much testosterone, she develops male-pattern hair growth, including

• facial hair,
• chest hair, and
• receding hairline.

The physique and facial features may also become much more masculine.

Men with estrogen-producing tumors may experience breast enlargement (gynecomastia). Libido is also usually reduced.

Testosterone-producing tumors are common, while estrogen-producing tumors are almost always malignant.

Adrenalectomy for Catecholamine-Producing Tumors

Tumors of the adrenal medulla are called pheochromocytomas. They release increased levels of catecholamines, namely

• adrenaline,
• norepinephrine
• and dopamine.

These hormones increase blood pressure and cardiac output. An excessive and uncontrolled release of these hormones can therefore lead to life-threatening hypertensive crises.

A characteristic symptom of these tumors is therefore high blood pressure. This can be persistently elevated. However, episodes of high blood pressure that occur in fits—so-called hypertensive crises—are typical. These are often accompanied by

• palpitations,
• marked pallor,
• cold sweats, and
• headaches.

If the tumor has not yet been diagnosed, these patients are at particularly high risk in stressful situations, such as

• in traffic accidents,
• childbirth, or
• surgery.

Adrenalectomy for Malignant Adrenal Tumors

Adrenal cortical carcinoma is a rare tumor with a poor prognosis. Early detection of the tumor is therefore of the utmost importance.

Malignant adrenal tumors are usually relatively large at the time of diagnosis. The larger a tumor is, the greater the risk that it is malignant. For this reason, nearly all adrenal tumors larger than 4 to 5 cm should be removed. Exceptions include adrenal cysts and so-called angiomyolipomas.

If a CT or MRI scan shows that the suspicious tissue contains little fat, the suspicion of a tumor increases. By injecting a contrast agent into a vein, a CT scan can reveal how blood flows into and out of a tumor. This can also provide clues as to whether the tumor is benign or malignant. In this way, even smaller tumors can be identified as suspected carcinomas.

About half of adrenal cortical carcinomas lead to increased hormone production. Tumors that produce excessive amounts of cortisone or testosterone are often carcinomas. Tumors that produce excessive amounts of estrogen or release various hormones in increased amounts are almost always malignant.

Metastases from other tumors often form in the adrenal glands. This means that primary tumors, such as

• bronchial carcinomas,
• melanomas, or
• renal cell carcinomas

have spread to the adrenal glands via the bloodstream or lymphatic system. Secondary tumors—or metastases—then form there.

In some cases, metastases are found in only one or both adrenal glands. In these cases, removal of the affected adrenal gland(s) through an adrenalectomy may be helpful.

Various surgical approaches and techniques are available for an adrenalectomy.

Generally, a distinction is made between open and endoscopic surgical procedures. In recent years, endoscopic surgery has become increasingly common. This procedure offers several advantages for the patient.

Endoscopic Surgical Technique for an Adrenalectomy

Benign tumors are generally removed using minimally invasive techniques today. Two different approaches are used:

• the transperitoneal approach with the patient in the lateral decubitus position
• the retroperitoneoscopic approach with the patient in the prone position.

It has not yet been determined whether one of these approaches is superior to the other.

Open Surgical Procedure for Adrenalectomy

Conventional surgical procedures are now used only for

• extremely large tumors and
• malignant adrenal tumors

.

The surgeon accesses the surgical site either through

• an incision in the abdominal cavity (laparotomy) or
• through a combined incision in the chest and abdomen (thoracoabdominal incision).

The access must be large enough to allow for safe manipulation of the surgical site. A clear view of the surgical site is crucial when removing malignant tumors. Damage to the tumor always leads to the spread of tumor cells. In such cases, a cure for the patient is highly unlikely.

The removal of an adrenal gland is a relatively safe procedure. Nevertheless, complications are possible during an adrenalectomy. To minimize the risk of complications as much as possible, thorough preparation of the patient is absolutely essential.

Preventing Complications During an Adrenalectomy

In cases of severe Cushing’s syndrome, it may be advisable to suppress cortisol production. To this end, the patient is pretreated with appropriate medications. Electrolyte levels must also be well-controlled prior to adrenalectomy.

Patients with Cushing’s syndrome are at particularly high risk for thrombosis and infection. They must therefore undergo thrombosis prophylaxis and antibiotic prophylaxis.

Patients with Conn’s syndrome should receive pretreatment with an aldosterone antagonist (e.g., Aldactone). This usually allows blood pressure to be controlled relatively well. Furthermore, potassium deficiency must be corrected before adrenalectomy.

Patients with a pheochromocytoma are at particular risk due to uncontrolled releases of catecholamines during adrenalectomy. Manipulation of the tumor, in particular, can trigger the release of these hormones. Therefore, the effects of catecholamines must be blocked in these patients.

There are medications that block the relevant receptors in the body. Patients must take the medication for 10 to 14 days prior to adrenalectomy. During this time, patients must also drink plenty of fluids. This helps them compensate for the significant fluid deficiency caused by the pheochromocytoma.

Surgical Complications of Adrenalectomy

Surgical complications are rare during an adrenalectomy. Bleeding may occur due to injury to adjacent large blood vessels. This bleeding can also become life-threatening.

Furthermore, adjacent organs may be injured, such as

• the pancreas,
• the spleen,
• the liver, and
• the kidneys.

What happens after an adrenalectomy depends on the reason for removing the adrenal gland.

Patients with Cushing’s syndrome require cortisone after the procedure. Cortisone-producing adrenal tumors suppress cortisol production in the healthy adrenal gland.

It can take months or even years for the healthy adrenal gland to recover its function. Cortisone replacement therapy must be continued until then. Otherwise, a life-threatening cortisol deficiency (Addisonian crisis) may occur. If both adrenal glands have been removed, the patient must take cortisol for the rest of their life. The patient should also take a mineralocorticoid (fludrocortisone).

Patients with Conn’s syndrome can usually discontinue some of their blood pressure medications after a successful adrenalectomy. The medications should be tapered off gradually under close monitoring of blood pressure levels.

Chemotherapy with mitotane may be necessary following the removal of an adrenal carcinoma. This decision should be made in close consultation with an experienced endocrinologist.

In addition, the patient should attend regular follow-up appointments after adrenalectomy.

Various conditions may necessitate the removal of the adrenal glands. Accurate hormonal testing is a prerequisite for adrenalectomy. This allows physicians to identify the different hormonal disorders.

The adrenalectomy itself is a relatively straightforward surgical procedure. Today, it is usually performed using minimally invasive techniques. The risks are also low if the patient is adequately prepared for the surgery.

Adrenal carcinomas are rare tumors. They have a poor prognosis, which depends crucially on the quality of the surgery. These tumors should therefore be treated at endocrine surgery centers.