Phaeochromocytoma Flashcards Preview

Internal Medicine > Phaeochromocytoma > Flashcards

Flashcards in Phaeochromocytoma Deck (9)
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1

Definition

Tumor arising from catecholamine-producing chromaffin cells of adrenal medulla

2

Key diagnostic factors

presence of risk factors
headache
palpitations
diaphoresis
FHx endocrine disorders/associated cancer syndromes->thyroid, MEN 2A, neurofibromatosis, von-Hippel lindau
hx prior phaeochromocytoma
hypertension
hypertensive retinopathy
tachyarrhythmias and myocardial infarction
pallor
impaired glucose tolerance/diabetes mellitus
panic attacks or a 'sense of doom'

3

Rule of 10%

10% are malignany
10% are extra-adrenal
10% are bilateral
10% are familial

4

What is an important extra-adrenal location

Aortic bifurcation

5

Classic triad

Headache
Sweating
Tachycardia

6

Investigations

*24 hour collection for catecholamines, metaneprhines, normetaneprhines and creatinine->elevated, at least 2-3 X normal for all measurement
*Serum free metanephrine and normetanephrine->elevated
*Plasma catecholamines ->may be elevated
*Genetic testing->may reveal familial disorders->VHL, MEN 2, NF
*FBC->erythocytosis
*Calcium->+
*Potassium->hypokalemia
*I-123 MIBG has a similar structure to noradrenaline and so incorporates into neurosecretory granules of the phaeochromocytoma, creating an image.

MRI/CT abdomen and pelvis->increased attenuation on non-enhanced CT. High signal on T2 weighted

7

Management

Hypertensive crisis->phentolamine

W/O:
Phenoxybenzamine (a-blocker) + atenolol/metoprolol (after alpha blockade to prevent tachy and arrythmias)
Hydration + high salt diet (>5g/day)
+/- CCB Nifedipine

In benign tumor->following medical management->
Surgical excision
If malignant following medical->Surgical debulking, chemotherapy, nuclear/radiation or ablation

8

Follow up

Assess metanephrines and normetaneprhines 1 week to 10 days postoperatively
Yearly f/u for at least 10 years->malignant potential may occur late
Seek medical help if symptoms recur

9

Complications

Acute HTN crisis
Neurological complications
Postoperative-> avoid complications with IV fluid