ADRENAL TUMOURS & ADRENALECTOMY

What are adrenal tumours?

Adrenal tumours are benign or malignant growths that develop in the adrenal glands.

The adrenal glands are small triangular glands located above the right and left kidneys. The adrenal glands comprise two distinct sub-organs, the cortex and the medulla.

The medulla produces and secretes adrenaline (epinephrine) and noradrenaline, which are essential for normal physiological control of cardiovascular function.

The cortex produces several hormones that are also essential for normal physiological function. These include cortisol, aldosterone and sex hormones.

Adrenal tumours may produce excessive amounts of these hormones, which can cause serious pathophysiological effects. Tumours that secrete excessive noradrenaline and/or adrenaline are known as phaeochromocytomas and can cause very serious high blood pressure, cardiac rhythm, and rate abnormalities. Adrenal tumours that produce excessive aldosterone cause high blood pressure and low blood levels of potassium ions, which is known as Conn’s Syndrome. Tumours that secrete excessive amounts of cortisol cause a constellation of effects known as Cushing’s Syndrome, which includes high blood pressure, rapid (typically central) weight gain with abnormal hair growth and purple “stretch marks” on the abdominal skin, diabetes, fragility of bones and other tissues and immune compromise. Tumours that produce excessive sex hormones can result in feminisation or masculinization effects. Many adrenal tumours are discovered incidentally on scans done for other reasons and do not secrete excessive amounts of hormones. However, sometimes they can grow large enough to exert pressure on adjacent organs. Also, when so-called non-functioning tumours are large, there is a greater risk that these could be primary carcinomas (cancers) of the adrenal glands.

Adrenal tumours are sometimes caused by some inherited genetic disorders.

Adrenal tumours that are small and discovered incidentally during imaging studies conducted for unrelated health concerns do not need to be removed.

Symptoms

Although adrenal tumours do not always produce symptoms, the following signs are considered clinical warning indicators:

• Hypertension
• Lethargy
• Unexplained changes in weight
• Quick heartbeat
• Changes to the blood sugar level
• Headaches

The aforementioned signs are general and warrant further evaluation by a qualified medical professional, such as Dr Stapleton.

Treatment

Treatment is determined by the classification of the tumour. The primary treatment modalities include the following:

• Surgery is commonly employed for malignant tumours or those causing excessive hormone production.
• Pharmacological therapy to regulate hormonal balance or manage related symptoms.
• Chemotherapy is typically reserved for advanced stages of malignant disease.
• Active surveillance is appropriate for small, non-functional tumours that require ongoing monitoring and follow-up assessments.

ADRENALECTOMY

Adrenalectomy is the operation to remove an adrenal gland, usually for a tumour arising from the adrenal gland.

Adrenal tumours may produce excessive amounts of adrenal hormones (functioning tumours), but some (non-functioning) tumours do not secrete excessively.

Pheochromocytomas, which arise from the adrenal medulla, produce excessive amounts of adrenaline and noradrenaline , which may cause severe hypertension (high blood pressure) with risks of cerebrovascular accidents (strokes), renal (kidney) damage and cardiac (heart) damage (rhythm disorders and cardiomyopathy). Tumours arising from the adrenal cortex may secrete excessive amounts of cortisol (Cushing's syndrome), aldosterone (Conn's Syndrome) or sex hormones.

Tumours that do not secrete excessive amounts of hormones may be detected incidentally by scans done for other reasons ("Incidentalomas"), or they may be highly malignant tumours that present with symptoms caused by tumour growth and invasion.

Adrenalectomy may be done laparoscopically or by open surgery, depending on the size and type of tumour. Patients with functioning tumours can have highly complex metabolic problems, which need to be carefully managed before, during and after the surgery to remove these tumours. Optimal management is done in close collaboration with endocrinologists, anaesthesiologists and intensive care physicians. Patients with pheochromocytoma need to have pre-operative preparation with complete α-blockade and sometimes β-blockade too. During surgery for pheochromocytomas, the surgeon has to be very careful not to handle the tumour excessively because of the risk of sudden, rapid release of adrenaline and noradrenaline into the circulation. The surgeon "dissects the patient off the tumour" and tries to clamp or ligate the adrenal vein as early as possible to minimise systemic hormonal release, but the anaesthesiologist needs to have measures in place to control blood pressure from minute to minute. Another problem with removing functioning tumours is that the excessive secretion by the tumour results in suppression of the other, normal, adrenal gland, so that after removal of the tumour, the patient suddenly experiences very subnormal levels of hormones, and the endocrinologist needs to administer the relevant hormones until the suppressed adrenal gland regains function. Patients with Cushing's syndrome are usually very obese and have very poor healing ability, so resecting these tumours laparoscopically is highly desirable, especially as the tumours are usually quite small. The tumours of Conn's syndrome are also preferably removed laparoscopically. On the contrary, open surgery may be preferred for large malignant tumours and for pheochromocytomas, 10% of which are malignant, and they are often very large, too. It may be easier to minimise tumour handling and safer to remove these at open surgery.

Furthermore, pheochromocytomas are bilateral and multiple in about 10% of cases and dealing with those situations would usually be simpler by open laparotomy.