Phaeochromocytoma

Question 1

What is a phaeochromocytoma?

Answer 1

It is a type of tumour that occurs in the adrenal medulla.

It is known as the 10% tumour as:
10% are extra-adrenal
10% malignant 
10% multiple
10% hyperglycaemic

Question 2

What symptoms do you get with phaeochromocytoma?

Answer 2

headache
sweating
palpitations
tremor
anxiety
hypertension (persistent in 70% of patients)

Question 3

What hormones are involved in phaeochromocytoma?

Answer 3

Catecholamines: epinephrine and norepinephrine (adrenaline and noradrenaline).
These are stress hormones, so cause an increase in BP, increase heart rate and breathing.

Question 4

What proportion of these tumours are inherited/genetic?

Answer 4

~30%

Question 5

Some genetic syndromes/ condtions are associated with phaeochromocytoma as part of them, what are these?

Answer 5

1in3 are genetic

Men II: phaeochromocytoma, meduallary thyroid cancer and parthyroid adenoma

Men III: phaeochromocytoma, meduallary thyroid cancer, mucocutaneous syndromes, multiple neuromas, marfanoid habitus (intellutual defect, along with flat face), hypertrophied corneal nerves

Von Huppel-Landau syndrome: phaeochromocytoma, cerebral and retinal angiomatosis, pancreatic and renal cysts, epidydmal cystadenomas (begnin tumours in the epididymus behind the testis)

Question 6

What tests are done?

Answer 6

24hr urine total metanephrine levels (catecholamines+ metanephrines)
MRI or CT

Question 7

Treatment?

Answer 7

Surgical removal

Question 8

Who gets phaeochromocytomas?

Answer 8

Usually aged between 20-50 years, both male and females can get it and they are usually benign but can be cancerous (1 in 10).