Adrenal Disease - Phaeochromocytoma

Question 1

Where is Adrenaline produced?

Answer 1

Chromaffin cells in the adrenal medulla of the adrenal glands.

Question 2

What is a Phaeochromocytoma?

Answer 2

A tumour of the chromaffin cells that secretes unregulated and excessive amounts of Adrenaline.

Question 3

What is Adrenaline?

Answer 3

A catecholamine hormone and neurotransmitter that stimulates the SNS.

Question 4

Associations of Phaeochromocytoma (3).

Answer 4

1. MEN II - 25% are familial.
2. Neurofibromatosis.
3. von-Hippel Lindau Syndrome.

Question 5

10% Rule (3).

Answer 5

1. Bilateral.
2. Cancerous.
3. Extra-Adrenal (commonest : adjacent to bifurcation of aorta).

Question 6

Clinical Presentation of Phaeochromocytoma (5).

Answer 6

1. Anxiety.
2. Sweating.
3. Headache.
4. Hypertension.
5. Palpitations, Tachycardia, Paroxysmal AF.
   * fluctuating when tumour secretes adrenaline.

Question 7

Investigations of Phaeochromocytomas (2).

Answer 7

1. 24 Hour Urine Catecholamine.

2. Plasma Free Metanephrine (breakdown product with a longer half-life than Adrenaline).

Question 8

Management of Phaeochromocytoma (3).

Answer 8

1. Alpha Blockers - Phenoxybenzamine.
2. B-Blockers after Established on A-Blockers.
3. Adrenalectomy - definitive management.
4. Control medically before surgery.