Phaeochromocytoma Flashcards Preview

Y3 - Conditions and prodedures > Phaeochromocytoma > Flashcards

Flashcards in Phaeochromocytoma Deck (6)
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1
Q

Define

A

• Catecholamine-producing tumours that usually arise from chromaffin cells of the adrenal medulla but are extra-adrenal in about 10% of cases.
o 10% are bilateral
o 10% are malignant
o Extra-adrenal phaeochromocytomas are referred to as paragangliomas

2
Q

What’s the aetiology/risk factors?

A

• Sporadic cases are of unknown aetiology
• Familial in up to 30%
• Familial cases are seen in patients with:
o Multiple Endocrine Neoplasia 2a and 2b
o von Hippel-Lindau syndrome
o Neurofibromatosis type 1

3
Q

Epidemiology?

A
  • RARE

* < 1% of hypertensive patients

4
Q

What are the presenting symptoms?

A
•	PAROXYSMAL episodes 
•	Headache (due to malignant hypertension)
•	Sweating 
•	Cardiorespiratory Symptoms
o	Palpitations 
o	Chest pain 
o	Dyspnoea 
•	GI Symptoms
o	Epigastric pain 
o	Nausea 
o	Constipation
•	Neuropsychiatric Symptoms
o	Weakness 
o	Tremor 
o	Anxiety
5
Q

What are the signs?

A
  • Hypertension
  • Postural hypotension
  • Pallor
  • Tachycardia
  • Fever
  • Weight loss
  • retinopathy
6
Q

What are the appropriate investigations?

A

• 24 hr urine collection - check for catecholamine levels (and check for fractionated metanephrine levels and creatinine levels
o NOTE: metanephrines are metabolites of adrenaline
• Plasma free metanephrines
• Screen for associated conditions
• Genetic testing (may reveal familial disorders)
• Tumour localisation (MRI or CT)
• I-MIBG scintigraphy (another way of visualising the tumour, high uptake intensity)