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Phaeochromocytoma

Title               

Ñ    Phaeochromocytoma

Definition

Ñ    Tumour of the sympathetic nervous system

Pathogenesis

Origins

Ñ    90% adrenal medulla

Ñ    10% sympathetic chain

Ñ    25% multiple

Ñ    10 % malignant

Associations

Ñ    MEN 2

Ñ    Neurofibromatosis

Ñ    von Hippel-Lindau syndrome

Pathophysiology

Ñ    Most tumours release:

Noradrenaline and adrenaline

Ñ    Large tumours and extra-adrenal lesions:

Noradrenaline

Clinical Features

History

Ñ    Intermittent features:

Usually

Ñ    Features may be constant

E&M

Ñ    Weight loss

IS

Ñ    Sweating

Ñ    Flushing

CVS

Ñ    Palpitations

Ñ    Chest pain

Ñ    Raynaud’s phenomenon

GIT

Ñ    Nausea and vomiting

Ñ    Constipation or diarrhoea

KUS

Ñ    Polyuria

Ñ    Nocturia

CNS

Ñ    Anxiety

Ñ    Panic attacks

Ñ    Headaches

Ñ    Tremor

Examination

E&M

Ñ    Fever

IS

Ñ    Pallor

Ñ    Flushing

CVS

Ñ    Hypertension

Ñ    Orthostatic hypotension

Ñ    Tachycardia

Ñ    Bradycardia

Investigations

Fluids

Urine

Ñ    Glycosuria

Ñ    Vanillyl mandelic acid:

Increased

Ñ    Metanephrines:

Increased

Ñ    Catecholamines:

Increased

Blood

Biochemistry

Ñ    Plasma catecholamines:

Increased

Imaging

CT, MRI

Ñ    Localise tumour

Nuclear Medicine

mIBG scan (I¹³¹ metaiodobenzylguanidine)

Ñ    Demonstrate ectopic site

Physiological studies

Ñ    Clonidine suppression test

Ñ    Glucagon stimulation test

Management

Control

Drugs

Ñ    Alpha blockade:

·          Phenoxybenzamine

·          Phentolamine:

Intravenous infusion, used during surgery

Ñ    Beta blockade:

Propranolol

Ñ    Alpha blockade before beta blockade

Ñ    Nitroprusside:

·          Severe hypertension:

During surgery

Surgery

Ñ    Remove tumour

Prevention

Follow up

Ñ    Clinical and biochemical review

Ñ    At least annual catechol excretion test

Prognosis

Ñ    95 % 5-year survival in non-malignant tumours

Ñ    10 % recurrence or develop second tumour

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