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year two - endocrine system > Phaeochromocytoma > Flashcards

Phaeochromocytoma Flashcards

1
Q

What is phaeochromocytoma?

A

A rare, benign tumour of the chromaffin cells in the adrenal gland

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2
Q

What is the function of chromaffin cells?

A

The secretion of adrenaline

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3
Q

How do phaeochromocytoma’s affect adrenaline levels?

A

It results in excessive production of adrenaline

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4
Q

What is adrenaline?

A

It is a catecholamine hormone and neurotransmitter that stimulates the sympathetic nervous system

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5
Q

What pattern do phaeochromocytoma’s follow?

A

10% rule

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6
Q

Describe the 10% pattern rule that phaeochromocytoma’s follow

A

10% Bilateral

10% Malignant

10% Extra-Adrenal

10% Hyperglycaemia

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7
Q

What are the two risk factors associated with phaeochromocytoma?

A

Genetics

Familial syndromes

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8
Q

What four genes are associated with phaeochromocytoma?

A

SDHD Gene

RET Gene

VHL Gene

NF1 Gene

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9
Q

What four familial syndromes are associated with phaeochromocytoma?

A

Multiple Endocrine Neoplasia Type 2 (MEN 2)

Von Hippel-Lindau Disease

Neurofibromatosis One (NF1)

Hereditary Paraganglioma Syndromes

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10
Q

What is MEN 2 disease?

A

MEN 2 is a disorder that results in tumours in more than one part of the endocrine system

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11
Q

What is Von Hippel-Lindau disease?

A

Von Hippel-Lindau disease can result in tumours at multiple sites, including the CNS, endocrine system, pancreas and kidneys

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12
Q

What is neurofibromatosis one (NF 1)?

A

Neurofibromatosis results in multiple tumours in the skin (neurofibromas), pigmented skin spots and tumours of the optic nerve

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13
Q

What are hereditary paraganglioma syndromes?

A

Hereditary paraganglioma syndromes are inherited disorders that result in either phaeochromocytomas or paragangliomas

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14
Q

Describe the cycle of phaeochromocytoma clinical features

A

Phaeochromocytoma results in adrenaline being secreted in bursts, giving periods of worst periods followed by more settled periods

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15
Q

What are the seven clinical features of phaeochromocytoma?

A

Hypertension

Headache

Sweating

Palpitations

Tremor

Pallor

Panic Attacks

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16
Q

Describe the presentation of hypertension in phaeochromocytoma patients

A

It is usually persistent, however, in some cases patients present with paroxysmal attacks of hypertension where bending down, coughing, etc increases their blood pressure

17
Q

What are the four investigations used to diagnose phaeochromocytoma?

A

24 Hour Urine Test

Blood Test

CT Scan

M-Iodobenzylguanidine (MIBG) Scan

18
Q

What is the gold standard investigation used to diagnose phaechromocytoma?

A

24 hour urine test of metanpehrines

19
Q

How do we conduct the 24-hour urine test used to diagnose phaeochromocytoma?

A

In this test, the patient collects a urine sample every time they urinate during a 24-hour period

We then measure the level of catecholamine hormones and metanephrines within these samples

20
Q

Why is the urine test for phaeochromocytoma conducted over 24 hours?

A

This is due to the fact that adrenaline levels fluctuate throughout the day and therefore a single result would be difficult to interpret

21
Q

What is metanephrine?

A

A breakdown product of adrenaline

22
Q

What specific blood test do we conduct to diagnose phaeochromocytoma? What result indicates phaeochromocytoma?

A

We measure the metanephrine plasma levels

If the plasma levels of metanephrine are elevated then a diagnosis is indicated

23
Q

What is a MIBG scan?

A

A MIBG scan involves injecting patients with radioactive iodine-123, which is then taken up by the phaeochromocytoma and appears on nuclear imaging.

24
Q

How do we treat phaeochromocytoma patients preoperatively?

A

We prescribe alpha blockers (phenoxybenzamine) and beta blockers (labetalol) drugs for seven to ten days before surgery

25
Q

Why do we treat phaeochromocytoma patients preoperatively?

A

It lowers the risk of patients developing dangerously low blood pressure during surgery

26
Q

What surgery is used to treat phaeochromocytoma patients?

A

Adrenalectomy

27
Q

What is adrenalectomy?

A

Adrenalectomy involves surgical removal of the entire adrenal gland with a phaeochromocytoma

28
Q

What are the three clinical features of MEN2a?

A

Medullary thyroid cancer

Hypercalcaemia

Phaeochromocytoma

29
Q

What are the three clinical features of MEN2b?

A

Medullary thyroid cancer

Marfonoid body habitus

Phaeochromocytoma

year two - endocrine system (17 decks)

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