Phaeochromocytoma Flashcards

1
Q

Define phaeochromocytoma

A

Tumour of the adrenal medulla that secretes catecholamines (NA/A), arising from chromaffin cells.

May also be extra-adrenal, found by the aortic bifurcation

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2
Q

Aetiology of phaeochromocytoma

A

Sporadic

40% hereditary in adults e.g. MEN-2A/2B, neurofibromatosis, Von Hippel Lindau syndrome

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3
Q

What is the 10% triad for phaeochromocytoma

A

10% extra-adrenal
10% malignant
10% bilateral

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4
Q

Classic triad of phaeochromocytoma

A

Headache
Sweating
Tachycardia

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5
Q

Presenting symptoms of phaeochromocytoma

A
Headache
Diaphoresis 
Palpitations 
Panic attack/sense of doom/anxiety/hyperactivity/confusion
Diarrhoea
Fever
Syncope
Angina
Dyspnoea
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6
Q

Signs of phaeochromocytoma

A
Hypertension (paroxysmal)
Tachycardia
Pallor
Retinopathy 
Orthostatic hypotension
Hypercalcaemia
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7
Q

Investigations of phaeochromocytoma

A

24h urine for metanephrines and metadrenaline: elevated

Serum free metanephrines and normetenephrines: elevated

Abdominal CT/MRI for tumour imaging: tumour

MIBG: high uptake intensity

Plasma catecholamine: elevated

Genetic testing for VHL syndrome, MEN1 and MEN2

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8
Q

Treatment for phaeochromocytoma

A
  1. Alpha blocker with IV fluid e.g. phenoxybenzamine
  2. Beta blocker e.g. atenolol
  3. laparascopic adrenalectomy
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