Phaeochromocytoma Flashcards
What is a Phaeochromocytoma?
A phaeochromocytoma is a rare tumour of chromaffin cells (usually in the adrenal medulla) that secretes catecholamines. However, occasionally extra-adrenal pheochromocytoma’s (paragangliomas) occur
The main Catecholamines are adrenaline, noradrenaline and dopamine and are involved in the fight or flight response
What will you find on a history taking of a patient with Phaeochromocytoma?
Symptoms: Intermittent Symptoms, can be multiple times a day or as little as monthly and last from seconds to hours Headache Sweating Palpitations Hypertension Tremor Anxiety Diabetes Patients may show typical cardiovascular, cerebrovascular, and renal effects of prolonged hypertension, such as congestive heart failure with pulmonary oedema.
Risk Factors:
30-50 Year olds
Multiple endocrine neoplasia 2A/B
Specific Questions to ask:
Ask how the symptoms have progressed over time – As more tumours grow symptoms worsen and become more frequent
Family history of phaeochromocytoma, Multiple endocrine neoplasia or neurofibromatosis
Think specifically in patients with treatment resistant hypertension (>3 drugs) or blood pressure that fluctuates rapidly
Differentials:
Essential hypertension – Are often asymptomatic and will be treated with first line anti hypertensives
Illegal Drug use – Cocaine
Alcohol Withdrawal – History of alcoholism
Anxiety disorder
Hyperthyroidism
What will you find on examination of a patient with Phaeochromocytoma?
End of the bed:
Neurofibromas and café au lait spots – Indicate neurofibromatosis
Weight loss
Hands:
Hypertension - paroxysmal in 50% of cases
Postural hypotension
Tremor
Chest:
Tachyarrhythmias – Commonly Sinus Tachycardia
Dilated or Hypertrophic Cardiomyopathy
Cardiac Failure as a result of chronic hypertension - Pulmonary oedema
Face:
Hypertensive retinopathy
Chest:
hypertension
What investigations will you order in suspected Phaeochromocytoma?
Bedside:
24-hour urine collection -catecholamines (adrenaline/noradrenaline), vanillylmandelic acid (VMA’s - end stage metabolite of catecholamines) and metanephrines (metabolite of adrenaline) should be measured
Capillary Glucose – Can be raised
Full set of observations
Bloods:
Serum Calcium - may be elevated
Serum metanephrines can be used – This test is less specific but easier to do, so can be used as a first line screening test
Haemoglobin -elevated due to reduction in circulating volume.
Plasma catecholamines and plasma metanephrines - Raised
Imaging: Finding the tumour
CT chest, abdo, pelvis - Extra-adrenal pheochromocytoma’s develop in chromaffin tissue of the sympathetic nervous system and can occur anywhere from the base of the brain to the urinary bladder. But normally all occur in the adrenal gland
PET scan may be used if unable to find the tumour with a CT
Special Tests:
Genetic Testing – Only once a diagnosis is made to look for MEN and Neurofibromatosis
What is the treatment of Phaeochromocytoma?
Medical:
Pre-operative treatment with alpha-blockers and beta-blockers is required to control blood pressure. Calcium channel blockers may be needed if these do not control it
Surgical:
Tumour Resection
What are the causes of a Phaeochromocytoma?
Idiopathic
Familial
Multiple Endocrine Neoplasia type 2A/B
Neurofibromatosis – Autosomal dominant condition causing many nerve tumours to grow
