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Benign tumours of the adrenal gland

A benign tumour of the adrenal gland is a non-cancerous growth that does not spread (metastasize) to other parts of the body and is not usually life-threatening. Most tumours of the adrenal gland are benign.

Functioning tumours produce excessive amounts of hormones normally produced by the adrenal gland. Non-functioning tumours do not produce hormones.

Functioning benign adrenal gland tumours do not spread from the adrenal gland. They can cause significant health problems because of the large amounts of hormone they produce.

Adrenal cortical adenoma

Adrenal cortical adenomas are the most common benign tumour of the adrenal gland. They start in the cortex (outer layer) of the adrenal gland. Adrenal cortical adenomas:

  • may be referred to as incidentalomas because they are often found when an ultrasound, CT scan or MRI of the abdomen is done for other health reasons
  • are small – usually less than 5 cm in diameter
  • usually occur in only one adrenal gland
  • usually secrete a single hormone – if functional

Risk factors

The following inherited or genetic disorders may increase a person’s chance of developing an adrenal cortical adenoma:

  • multiple endocrine neoplasia (MEN) syndromemultiple endocrine neoplasia (MEN) syndromeA rare genetic condition that is associated with tumours in more than one endocrine gland and an increased risk of developing endocrine system cancers. – type 1
  • McCune-Albright syndrome
  • Cowden syndromeCowden syndromeA genetic condition in which many non-cancerous (benign) growths (hamartomas) form in the skin, breast, thyroid, colon, intestines and inside the mouth.
  • Li-Fraumeni syndromeLi-Fraumeni syndromeA rare genetic condition that is associated with an increased risk of developing certain types of cancers, including breast cancer, brain tumours, acute leukemia, soft tissue and bone sarcomas and adrenal cortical carcinomas.

Signs and symptoms

Most people do not have signs and symptoms with adrenal cortical adenomas. If an adenoma is functioning, it may not produce enough excess hormones to cause symptoms. If excess hormones are produced by the adenoma, the hormone-related signs and symptoms are the same as those from malignant adrenal tumours. Some adenomas cause Conn syndrome and others cause Cushing syndrome.

Diagnosis

Many adrenal cortical adenomas are discovered by accident during an ultrasound or scan for other health reasons. The same diagnostic tests are done as for malignant tumours. Tests may include:

  • blood and urine tests to check hormone levels – to determine if the adenoma is functioning
  • computed tomography (CT) scan
  • magnetic resonance imaging (MRI)

Treatment

Treatment options for adrenal cortical adenomas may include:

  • observation
    • CT scans are used to observe growth of the adenoma.
    • Laboratory tests are used to look for changes in hormone levels.
    • If the adenoma remains small and doesn’t make any hormones, treatment may not be needed.
  • surgery to remove the adrenal gland that contains the adenoma
    • The adrenal gland may be removed if the adenoma is large, increases in size or is making hormones.
  • medications that block the production or actions of the hormones
    • Drugs that effectively treat hormone-related symptoms may be a treatment choice for people who cannot have surgery because of other health problems.

Pheochromocytoma

Pheochromocytomas are the most common type of tumour that starts in chromaffin cells of the medulla (inner layer) of the adrenal gland.

  • A small number of pheochromocytomas start outside the medulla of the adrenal gland. These are called extra-adrenal pheochromocytomas or paragangliomas.
  • Most pheochromocytomas are benign. However, they overproduce hormones (called catecholamines), which can cause serious health problems, such as high blood pressure and heart problems.
  • Pheochromocytomas occur most often between the ages of 29 and 50 years.
  • Benign pheochromocytomas and paragangliomas do not spread to other places, but they sometimes occur in both adrenal glands or in more than one area at the same time.
  • Approximately 25% of pheochromocytomas are found in people with certain hereditary conditions. Genetic testing may be suggested for people who develop a pheochromocytoma or paraganglioma.

Risk factors

The following inherited or genetic disorders may increase a person’s chance of developing a pheochromocytoma:

  • multiple endocrine neoplasia (MEN) syndromemultiple endocrine neoplasia (MEN) syndromeA rare genetic condition that is associated with tumours in more than one endocrine gland and an increased risk of developing endocrine system cancers. – type 2A and 2B
  • von Hippel-Lindau (VHL) syndromevon Hippel-Lindau (VHL) syndromeA rare genetic condition that affects blood vessels in the eyes, brain, spinal cord, adrenal glands or other parts of the body, making them grow abnormally.
  • neurofibromatosisneurofibromatosisA genetic condition that affects the nervous system. It affects the development and growth of neurons (nerve cells), causes tumours (neurofibromas) to grow on nerves and may produce other abnormalities in muscles, bones and skin. – type 1
  • succinate dehydrogenase (SDH) gene mutations

Signs and symptoms

The signs and symptoms of pheochromocytoma tumours are related to the excessive amounts of hormones produced by the tumour. Signs and symptoms are the same for both the benign and malignant forms of pheochromocytoma.

Diagnosis

Diagnostic tests are the same for both benign and malignant pheochromocytomas. Tests may include.

  • blood and urine tests to check hormone levels
  • computed tomography (CT) scan
  • magnetic resonance imaging (MRI)
  • metaiodobenzylguanidine (MIBG) scan

Treatment

Treatment options for benign pheochromocytomas may include:

  • medications before surgery to manage blood pressure and heart rhythms
  • surgery to remove the adrenal gland
    • Medications may be given to replace hormone levels following surgery and until levels return to normal. (Hormone levels usually return to normal within 2 weeks after surgery.)

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