Phaeochromocytoma

Question 1

What is a pheochromocytoma?

Answer 1

They are tumours affecting the adrenal medulla of the adrenal gland

Question 2

Why is phaeochromocytoma called the 10% tumour?

Answer 2

10% malignant 
10% bilateral
10% extra-adrenal 
10% familial 
10% associated with hyperglycaemia 
105 in children

Question 3

Presentation of phaeochromocytoma?

Answer 3

• Hypertension
• Headache
• Sweating
• Palpitations
• Breathlessness
• Constipation
• Anxiety/fear
• Weight loss
• Flushing-uncommon

Question 4

Classic triad of symptoms for phaeochromocytoma?

Answer 4

• Sweating
• Headache
• Hypertension

Question 5

Potential clues for phaeochromocytoma?

Answer 5

Labile HT
Postural HT
Paroxysmal sweating, headache, pallor, tachycardia

Question 6

Signs of phaeocrhomocytoma?

Answer 6

• HT
• Postural HT
• Pallor
• Bradycardia
• Tachycardia

Question 7

Biochemistry findings in phaeochromocytoma?

Answer 7

• Hyperglycaemia
• Low K level
• High haematocrit
• Mild hypercalcaemia
• Lactic acidosis

Question 8

How is phaeochromocytoma investigated?

Answer 8

• Confirm catecholamine XS
• Urine 2 x 24 hour
• Plasma (at time of symptoms)

Question 9

Diagnosis of phaeochromocytoma?

Answer 9

• Identify source of catecholamine XS
• MRI scan: abdo, whole body
• MIBG- Meta-iodobenzylguanidine
• PET scan

Question 10

Management of Phaeochromocytoma?

Answer 10

• Full a & b blockade (A before B)
• Fluid/blood replacement
• Surgical= excision
• Chemo for malignant
• Family tracing

Question 11

What drugs are used as a and b blockade in paheochromocytoma?

Answer 11

• Phenoxybenzamine (alpha blocker)

- Propanolol, atenolol or metoprolol (Beta blocker)

Question 12

Clinical syndromes associated with phaeochromocytomas?

Answer 12

• Multiple endocrine neoplasia 2 (MEN2)
• Von-Hippel-Lindau syndrome
• Succinate dehydrogenase mutations
• Neurofibromatosis
• Tuberose sclerosis

Question 13

Things to be aware of in phaeochromocytomas?

Answer 13

• Catecholamines raised in heart failure
• Dopamine > norepinephrine > adrenaline
• Remember genetic syndromes