Phaechromocytomas Flashcards
1
Q
Where do phaechromocytomas arise from?
A
the chromaffin cells of the adrenal medulla
2
Q
What are the symptoms of phaechromocytomas?
A
hypertension pyrexial sweating pallor tachycardia anxiety constipation postural hypotension
3
Q
What does the rule of 10% refer to in phaechromocytomas?
A
10%: familial malignant bilateral extra adrenal
4
Q
What are the coplications of phaechromocytomas?
A
left ventricular failure myocardial necrosis stroke shock parylitic ileus of the bowel
5
Q
What are the biochemical signs of phaechromocytomas?
A
decreased K increased Ca icreased Hb lactic acidosis hyperglycaemia
6
Q
How are phaechromocytomas diagnosed?
A
MRI scan of whole body and abdomen PET scan Metaiodobenzylguanidine scan (picks up neuroednocrine tumour cells)
7
Q
What is the treatment for phaeochromocytomas?
A
block alpha and beta receptors - ALPHA before BETA ( may cause vasoconstriction)
- phenoxybenzamide - alpha
- propanolol, atenolol, metoprolol - beta
Fluid/blood replacement
Laprascopic removal
Chemotherapy if malignant
genetic testing
8
Q
What gene mutations are phaechromocytomas associated with?
A
succinate dehydrogenase mutations MEN2 Von-Hippel Lindau syndrome Tuberous sclerosis Neurofibromatosis
