Phaeochromocytoma Flashcards
What is a pheochromocytoma?
They are tumours affecting the adrenal medulla of the adrenal gland
Why is phaeochromocytoma called the 10% tumour?
10% malignant 10% bilateral 10% extra-adrenal 10% familial 10% associated with hyperglycaemia 105 in children
Presentation of phaeochromocytoma?
- Hypertension
- Headache
- Sweating
- Palpitations
- Breathlessness
- Constipation
- Anxiety/fear
- Weight loss
- Flushing-uncommon
Classic triad of symptoms for phaeochromocytoma?
- Sweating
- Headache
- Hypertension
Potential clues for phaeochromocytoma?
Labile HT
Postural HT
Paroxysmal sweating, headache, pallor, tachycardia
Signs of phaeocrhomocytoma?
- HT
- Postural HT
- Pallor
- Bradycardia
- Tachycardia
Biochemistry findings in phaeochromocytoma?
- Hyperglycaemia
- Low K level
- High haematocrit
- Mild hypercalcaemia
- Lactic acidosis
How is phaeochromocytoma investigated?
- Confirm catecholamine XS
- Urine 2 x 24 hour
- Plasma (at time of symptoms)
Diagnosis of phaeochromocytoma?
- Identify source of catecholamine XS
- MRI scan: abdo, whole body
- MIBG- Meta-iodobenzylguanidine
- PET scan
Management of Phaeochromocytoma?
- Full a & b blockade (A before B)
- Fluid/blood replacement
- Surgical= excision
- Chemo for malignant
- Family tracing
What drugs are used as a and b blockade in paheochromocytoma?
- Phenoxybenzamine (alpha blocker)
- Propanolol, atenolol or metoprolol (Beta blocker)
Clinical syndromes associated with phaeochromocytomas?
- Multiple endocrine neoplasia 2 (MEN2)
- Von-Hippel-Lindau syndrome
- Succinate dehydrogenase mutations
- Neurofibromatosis
- Tuberose sclerosis
Things to be aware of in phaeochromocytomas?
- Catecholamines raised in heart failure
- Dopamine > norepinephrine > adrenaline
- Remember genetic syndromes