Phaeochromocytoma Flashcards
What?
Neoplasm derived from chromatin cells of adrenal medulla
Secretes catecholamines
rare
Catectholamines?
Usually adrenal glands send catecholamines into blood when physically or emotionally stressed
E.g. adrenaline, noradrenaline
Where do these arise?
In adrenal medulla
Associations?
Assoc. with MEN2 syndromes
Symptoms?
Anxiety/ panic attacks Palpitations Tremor Sweating Headache Flushing Nausea and/ or vomiting Weight loss Constipation or diarrhoea Raynaud’s Chest pain Polyuria/ nocturia Palpation of tumour
Signs?
Hypertension Tachycardia/ arrhythmias Bradycardia Orthostatic hypotension Pallor or flushing Glycosuria Fever
Complications?
Cardiac failure
Infarction
Arrhythmias
CVA
Investigations?
Measure urinary catecholamines and metabolites Resting plasma metanephrines are raised Plasma chromogranin A raised Clonidine suppression test CT/ MRI MIBG scanning Genetic testing
Diagnosis?
Detection of urinary excretion of catecholamines and metabolites
Management?
Removal of tumour if possible
Medical preoperative and preoperative treatment
What medical pre and preoperative treatment is used?
Complete alpha and beta blockade with phenoxybenzamine (20-80mg initially in divided dose)
Alpha must precede beta
Then propanolol (120- 240mg)
Intravenous hydration to re- expand contracted plasma volume
If operation not possible?
Combined alpha and beta- blockade used long term
What are these referred to as?
10% tumour
Management?
Removal of tumour if possible
Medical preoperative & preoperative treatment
Medical preoperative & preoperative treatment?
Complete alpha and beta blockade with phenoxybenzamine (20-80mg initially in divided dose) Then propanolol (120- 240mg) Intravenous hydration to re- expand contracted plasma volume
