Adrenal Tumours

Definition | Aetiology | Pathophysiology | Risk Factors | Signs and Symptoms | Investigations | Management | Example Management | References

Definition

Adrenal tumours are abnormal growths that develop in the adrenal glands, which are located above each kidney. These tumours can be benign (non-cancerous) or malignant (cancerous) and can affect hormone production, leading to various clinical syndromes.

Aetiology

The causes of adrenal tumours can vary:

• Adrenocortical Adenomas: Benign tumours often discovered incidentally during imaging for other conditions.
• Adrenocortical Carcinomas: Rare malignant tumours that may arise sporadically or in association with genetic syndromes.
• Phaeochromocytomas: Tumours arising from the adrenal medulla, producing excess catecholamines.
• Metastatic Tumours: Secondary tumours that have spread from other parts of the body.

Pathophysiology

The pathophysiology of adrenal tumours depends on the type and location of the tumour:

• Adrenocortical Tumours: Can lead to overproduction or underproduction of adrenal cortex hormones such as cortisol, aldosterone, and androgens.
• Phaeochromocytomas: Cause excessive production of catecholamines (adrenaline and noradrenaline), leading to episodic or sustained hypertension and other symptoms.
• Metastatic Tumours: Can disrupt adrenal function and cause symptoms related to the primary cancer site.

Risk Factors

• Family history of adrenal tumours or related genetic conditions (e.g., Li-Fraumeni syndrome, MEN2)
• Genetic mutations (e.g., TP53, MEN1, RET)
• Age (most common in middle-aged to older adults)
• History of other cancers (for metastatic adrenal tumours)

Signs and Symptoms

Symptoms of adrenal tumours can vary depending on the type and hormonal activity:

• Functional Adrenocortical Tumours:
  • Cushing’s syndrome (excess cortisol): Weight gain, moon face, muscle weakness, hypertension, diabetes.
  • Primary hyperaldosteronism (excess aldosterone): Hypertension, hypokalaemia, muscle cramps.
  • Virilisation (excess androgens): Hirsutism, acne, menstrual irregularities.
• Phaeochromocytomas: Episodic headaches, palpitations, sweating, severe hypertension.
• Non-functional Tumours: Often asymptomatic and discovered incidentally.

Investigations

• Clinical history and physical examination
• Blood tests: To measure hormone levels (e.g., cortisol, aldosterone, catecholamines)
• 24-hour urinary hormone tests: To assess catecholamine and metanephrine levels for phaeochromocytomas
• Imaging studies: CT or MRI of the abdomen to identify and characterise adrenal masses
• Adrenal vein sampling: To differentiate between unilateral and bilateral adrenal disease in cases of primary hyperaldosteronism
• Genetic testing: For patients with a family history or clinical suspicion of a hereditary syndrome

Management

Primary Care Management

• Referral to endocrinologist: For evaluation and management of suspected adrenal tumours.
• Monitoring: Regular follow-up for patients with known benign adrenal masses to monitor for changes in size or function.
• Education: Informing patients about the potential symptoms of hormonal excess and the importance of follow-up.

Specialist Management

• Surgery: Adrenalectomy (removal of the adrenal gland) for functional tumours, malignant tumours, or large benign tumours.
• Medical therapy:
  • For Cushing’s syndrome: Medications to inhibit cortisol production (e.g., ketoconazole, metyrapone).
  • For primary hyperaldosteronism: Mineralocorticoid receptor antagonists (e.g., spironolactone) if surgery is not feasible.
  • For phaeochromocytomas: Alpha-blockers and beta-blockers to control hypertension pre-operatively.
• Radiotherapy or chemotherapy: For metastatic or unresectable adrenal cancers.
• Genetic counselling: For patients with hereditary syndromes associated with adrenal tumours.

Example Management for Adrenal Tumours

A patient diagnosed with a phaeochromocytoma presenting with episodic hypertension, palpitations, and sweating should be referred to an endocrinologist. Pre-operative management includes alpha-blockers (e.g., phenoxybenzamine) to control blood pressure and prevent hypertensive crises during surgery. Beta-blockers may be added after adequate alpha-blockade to manage tachycardia. Surgical removal of the tumour (adrenalectomy) is the definitive treatment. Post-operative monitoring of blood pressure and catecholamine levels is essential to ensure complete tumour removal and detect recurrence.

References

1. NICE. (2024). Adrenal Tumours: Diagnosis and Management. Retrieved from NICE
2. NHS. (2023). Adrenal Gland Tumours. Retrieved from NHS
3. European Society of Endocrinology. (2022). Guidelines for the Management of Adrenal Incidentalomas. Retrieved from European Society of Endocrinology
4. American Association of Clinical Endocrinologists (AACE). (2021). Clinical Practice Guidelines for the Diagnosis and Management of Adrenal Tumours. Retrieved from AACE

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