PhaeochromocytoPhaeochromocytomama

This us a rare catecholamine- This us a rare catecholamine- producing tumour, producing tumour,

and a treatable cause of hypertension and a treatable cause of hypertension (incidence <1%). (incidence <1%).

90% are in the adrenal medulla, 90% 90% are in the adrenal medulla, 90% benign . benign .

and these are usually unilateral 90%). and these are usually unilateral 90%).

Sometimes inherited - autosomal Sometimes inherited - autosomal dominant. dominant.

AssociatioAssociation: n: multiple endocrine neoplasia (Men Type Il). multiple endocrine neoplasia (Men Type Il).

Tumours outside the medulla may be in Tumours outside the medulla may be in

paraganglia ( = phaeochrome bodies, i.e paraganglia ( = phaeochrome bodies, i.e

collections of epinephirine - secreting collections of epinephirine - secreting

chromaffin cells) - typically by the aortic chromaffin cells) - typically by the aortic

bifurcation (organ of Zuckerkandl).bifurcation (organ of Zuckerkandl).

Symptoms and Symptoms and signs: signs:

Episodic hypertension, Episodic hypertension,

chest tightness, chest tightness,

restlessness, restlessness,

anxiety, anxiety,

pallor, pallor,

and weakness.and weakness.

Test: Test:

Glycosuria during attacks in 30%. Glycosuria during attacks in 30%.

Screening: Screening:

24h urine collection for24h urine collection for

4-OH-3 methoxymandelate (HMMA, 4-OH-3 methoxymandelate (HMMA,

VMA) or total ( or free) VMA) or total ( or free)

metadrenalines .metadrenalines .

Full Full investigations: investigations:

consult specialist centre: consult specialist centre:

consider MIB scan (metadrenalines consider MIB scan (metadrenalines

benzylguanidine) or clonidine benzylguanidine) or clonidine

suppression test & abdomen CT/MRI suppression test & abdomen CT/MRI

Scan.Scan.

Treatment Treatment Surgery: Surgery:

Careful BP control for 2 weeks Careful BP control for 2 weeks pre-alfa-blocker pre-alfa-blocker (phenoxybenzamine before B-(phenoxybenzamine before B-blockers (Propranolol). blockers (Propranolol).

Consult anaesthetist.Consult anaesthetist.

Post operative collection 24 h Post operative collection 24 h urine as above; monitor BP (risk urine as above; monitor BP (risk of BP↓↓)of BP↓↓)

The clinical features of The clinical features of PhaeochromocytomaPhaeochromocytoma

features including:features including:

• • Chest tightness Chest tightness • Skin mottling. • Skin mottling.

• • Abdominal pain Abdominal pain • Palpitations• Palpitations

• • Spots b the eSpots b the e • Weight loss• Weight loss

• • TremorTremor • Pallor• Pallor

• • Pins and needlesPins and needles • Dyspnoea• Dyspnoea

• • Cold feetCold feet • Claudicarion• Claudicarion

a Herninopiaa Herninopia

PurpuraPurpura

a Vomitinga Vomiting

• • FlushingFlushing

• • Pulsatile scotomasPulsatile scotomas

• • SweatingSweating

• • Faints (postural at drop)Faints (postural at drop)

Symptoms are precipitated by stretching, Symptoms are precipitated by stretching,

sneezing, sneezing,

stress, stress,

sex, sex,

smoking surgery, smoking surgery,

or parturiti by agents such as cheese, or parturiti by agents such as cheese,

alcohol, alcohol,

or the ti-i cyclic you so kindly prescribed, or the ti-i cyclic you so kindly prescribed,

thinking that the patient’s bizarre thinking that the patient’s bizarre symptoms were only explicable by symptoms were only explicable by depression.depression.

These causes may last for a few minutes These causes may last for a few minutes or a week. or a week.

Suddenly the patient may feel ‘as it Suddenly the patient may feel ‘as it about to die’—and then gets better, about to die’—and then gets better,

or goes on to stroke or cardiovascular or goes on to stroke or cardiovascular collapse. collapse.

On examination, there may be no signs, On examination, there may be no signs, or hypertension (± signs of or hypertension (± signs of

cardiomyopathy or heart failure) and cardiomyopathy or heart failure) and thyroid swelling (episodic) and thyroid swelling (episodic) and glycosuria during an attack—or glycosuria during an attack—or terminal haematuria from a bladder terminal haematuria from a bladder phaeochromocytoma.phaeochromocytoma.