Phaeochromocytoma Flashcards
Define Phaeochromocytoma?
Catecholamine-producing tumours that usually arise from chromaffin cells of the adrenal medulla but are extra-adrenal in about 10% of cases
What percentage of Phaeochromocytomas are bilateral and malignant?
10% are bilateral
10% are malignant
What are Extra-adrenal phaeochromocytomas referred to as?
Paragangliomas
What is the aetiology of Phaeochromocytoma?
Sporadic cases are of unknown aetiology
Familial in up to 30%
What conditions are Familial cases of Phaeochromocytoma seen in?
MEN2a
von Hippel-Lindau Syndrome
Neurofibromatosis type 1
What is the epidemiology of Phaeochromocytoma?
RARE
< 0.2 % of hypertensive patients
What are the general presenting symptoms of Phaeochromocytoma?
Paroxysmal Episodes
Headache (due to malignant hypertension)
Sweating
What are the Cardiorespiratory symptoms of Phaeochromocytoma?
Palpitations
Chest Pain
Dyspnoea
What are the GI symptoms of Phaeochromocytoma?
Epigastric pain
Nausea
Constipation
What are the Neuropsychiatric Symptoms of Phaeochromocytoma?
Weakness
Tremor
Anxiety
What are the signs of Phaeochromocytoma on physical examination?
Hypertension Postural hypotension Pallor Tachycardia Fever Weight Loss
What investigations would you do for Phaeochromocytoma?
24 hr urine collection Plasma free metanephrines Tumour localisation I-MIBG scintigraphy Screen for associated conditions Genetic testing
Why do we do 24 hr urine collection for Phaeochromocytoma?
Check for catecholamine levels (and check for fractionated metanephrine levels)
What are metanephrines?
Metabolites are adrenaline
How do we do Tumour Localisation in Phaeochromocytoma?
MRI or CT
