Phaeochromocytoma Flashcards

1
Q

What is a pheochromocytoma?

A

They are tumours affecting the adrenal medulla of the adrenal gland

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2
Q

Why is phaeochromocytoma called the 10% tumour?

A
10% malignant 
10% bilateral
10% extra-adrenal 
10% familial 
10% associated with hyperglycaemia 
105 in children
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3
Q

Presentation of phaeochromocytoma?

A
  • Hypertension
  • Headache
  • Sweating
  • Palpitations
  • Breathlessness
  • Constipation
  • Anxiety/fear
  • Weight loss
  • Flushing-uncommon
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4
Q

Classic triad of symptoms for phaeochromocytoma?

A
  • Sweating
  • Headache
  • Hypertension
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5
Q

Potential clues for phaeochromocytoma?

A

Labile HT
Postural HT
Paroxysmal sweating, headache, pallor, tachycardia

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6
Q

Signs of phaeocrhomocytoma?

A
  • HT
  • Postural HT
  • Pallor
  • Bradycardia
  • Tachycardia
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7
Q

Biochemistry findings in phaeochromocytoma?

A
  • Hyperglycaemia
  • Low K level
  • High haematocrit
  • Mild hypercalcaemia
  • Lactic acidosis
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8
Q

How is phaeochromocytoma investigated?

A
  • Confirm catecholamine XS
  • Urine 2 x 24 hour
  • Plasma (at time of symptoms)
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9
Q

Diagnosis of phaeochromocytoma?

A
  • Identify source of catecholamine XS
  • MRI scan: abdo, whole body
  • MIBG- Meta-iodobenzylguanidine
  • PET scan
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10
Q

Management of Phaeochromocytoma?

A
  • Full a & b blockade (A before B)
  • Fluid/blood replacement
  • Surgical= excision
  • Chemo for malignant
  • Family tracing
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11
Q

What drugs are used as a and b blockade in paheochromocytoma?

A
  • Phenoxybenzamine (alpha blocker)

- Propanolol, atenolol or metoprolol (Beta blocker)

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12
Q

Clinical syndromes associated with phaeochromocytomas?

A
  • Multiple endocrine neoplasia 2 (MEN2)
  • Von-Hippel-Lindau syndrome
  • Succinate dehydrogenase mutations
  • Neurofibromatosis
  • Tuberose sclerosis
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13
Q

Things to be aware of in phaeochromocytomas?

A
  • Catecholamines raised in heart failure
  • Dopamine > norepinephrine > adrenaline
  • Remember genetic syndromes
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