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Endocrinology Medicine > Phaeochromocytoma > Flashcards

Phaeochromocytoma Flashcards

1
Q

What is a phaeochromocytoma?

A

Benign tumour of chromaffin cells of adrenal medulla

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2
Q

What are the risk factors for phaeochromocytoma?

A

MEN 2a + 2b (multiple endocrine neoplasia)

Von-Hippel-Lindau disease

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3
Q

What are the conditions associated with MEN 1?

A

Pancreatic tumours
Pituitary adenoma
Parathyroid hyperplasia

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4
Q

What conditions are associated with MEN 2a?

A

Parathyroid hyperplasia
Medullary thyroid carcinoma
Phaeochromocytoma

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5
Q

What conditions are associated with MEN 2b?

A

Mucosal neuromas
Marfanoid body habitus
Medullary thyroid carcinoma
Phaeochromocytoma

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6
Q

What is the effected gene is MEN2a + MEN2b?

A

RET proto-oncogene

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7
Q

What is Von Hippel-Lindau disease?

A

Autosomal dominant condition with a germline mutation on in the VHL suppressor gene on chromosome 3

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8
Q

What are the other conditions associated with Von Hippel-Lindau disease?

A

Renal cell carcinoma
Cerebellar haemangioblastomas
Renal cysts
Pancreatic cysts

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9
Q

What are the clinical features of phaeochromocytoma?

A

Episodic

  • headaches
  • palpitations
  • hypertension
  • anxiety
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10
Q

What rule is associated with phaeochromocytomas?

A

Rule of 10s

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11
Q

What is the rule of 10s?

A

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

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12
Q

What investigations are indicated for a suspected phaeochromocytoma?

A

ECG
24 hour urine collection

Bloods

  • serum free metanephrines
  • plasma catecholamines
  • U+Es
  • bone profile

CT AP

Genetic testing

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13
Q

What are metanephrines?

A

Breakdown product of catecholamines

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14
Q

What special test can be done?

A

I-123 MIBG scintography

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15
Q

What is the management for phaeochromocytoma?

A

Alpha blockade (phenoxybenzamine)

2 weeks later start beta blockade (propranolol)

Adrenalectomy

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16
Q

Why is 2 weeks of alpha blockers given before beta blockers are started?

A

If beta blockers given first, lead to unopposed alpha stimulation

Alpha stimulation = vasoconstriction -> can worsen hypertension

17
Q

What are the complications of a phaeochromocytoma?

A

Hypertensive crisis

Post-op hypotension

18
Q

What are features of a hypertensive crisis?

A 
Encephalopathy 
MI 
Heart failure 
Cerebral haemorrhage
Arrhythmia
19
Q

How is a hypertensive crisis managed?

A

IV labetalol

20
Q

How is post-op hypotension managed?

A

IV fluids

Endocrinology Medicine (22 decks)

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