Phaetochromocytoma

Question 1

Define Phaechromocytoma

Answer 1

EMERGENCY-

Cancer of the medulla of the adrenal glands causing over producing adrenaline

Question 2

Signs and Sx of Phaechromocytoma

Answer 2

Nervousness, sweat
Racing heartbeat/palpitation
EPISODIC severe hypertension 
Headache
Panic attach
Pallor
Tremors
Thats a pheo crisis

severe vasoconstriction also causes ischemic damage

can find abdominal masses, papillodema, Fever, diarrhoea

Question 3

Aetiology and Risk factors of Phaechromocytoma

Answer 3

90% are Cancer of the chromaffin cells
Tend to be sporatic, but possible 40% are inherited + lots of genetic predispositions
And some genes straight up cause familial Pheo

risk factors
FHx
Neurofibromatosis Type 1, multiple endocrine neoplasia type 2a/2b
SDH mutation

Question 4

Epidiemology of Phaechromocytoma

Answer 4

Rare -0.6 in 100000
rare cause of secondary hypertension
(<1% of HTN cause)

1/3 are associated with other familial syndromes like Neurofibromatosis type 1

Question 5

Investigations of Phaechromocytoma

Answer 5

Blood pressure-super high, but transient

24h urine catecholamine-raised

Serum free metanephrine (precursors)-high
and can use scans that label the problem (MIBG)

Question 6

Management of Phaechromocytoma

Answer 6

alpha blockade is first port of call-emergency
then B blockers (always in that order)
Once blocked, they can be quite stable-
then its surgery (usually emergency surgery, but not always necessary)

Question 7

Complications of Phaechromocytoma

Answer 7

Acute hypertensive crisis-MI, arrythmias, stroke, acute heart failure

Neuro issues-epilepsy, altered mental state

malignancy

death if not treated

Question 8

Prognosis of Phaechromocytoma

Answer 8

Surgery is curative in 85% of cases
And Alpha blockade is also really good at calming any Sx until surgery
But often retain some HTN in the long run

Emergency–prognosis not great if not treated

can have some malignant cases-42% 5 year survival rate