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Endocrine- Adrenal > Tumours > Flashcards

Tumours Flashcards

1
Q

Adrenocortical tumours usually affect who?

A

Adults

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2
Q

Adrenocortical tumours which occur in children are usually in association with what?

A

Li-Fraumeni Syndrome which mutates p53

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3
Q

What is the only definite criteria for malignancy in adrenal tumours?

A

Metastases

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4
Q

Are adrenal adenomas differentiated or not?

A

They are differentiated

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5
Q

Which is more likely to be functional, an adrenal adenoma or carcinoma?

A

Carcinoma

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6
Q

Virilising tumours are more likely to be?

A

Malignant

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7
Q

Local invasion of an adrenal carcinoma will usually affect where?

A

Kidneys and retroperitoneum

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8
Q

Adrenal carcinomas spread through the blood. Where will this affect?

A

Lungs, liver, bone

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9
Q

What is the prognosis of adrenal carcinoma like?

A

Poor

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10
Q

Who does neuroblastoma occur in?

A

Children, usually diagnosed at around 18 months though can be earlier

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11
Q

What predicts a poorer outcome in neuroblastoma?

A

Amplification of n-myc and expression of telomerase

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12
Q

What is a phaeochromocytoma?

A

A neuroendocrine tumour derived from chromaffin cells of the adrenal medulla

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13
Q

What are phaeochromocytomas which occur outwith the adrenal medulla (elsewhere in the sympathetic chain) known as?

A

Paraganglioma

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14
Q

What are the 10% rules with phaeochromocytoma?

A

10% extra-adrenal, 10% bilateral, 10% malignant, 10% not associated with hypertension

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15
Q

When are up to 50% of cases of phaechromocytoma bilateral?

A

When they are familial

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16
Q

Malignant phaeochromocytomas are most common when?

A

They are extra-adrenal

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17
Q

Chromaffin cells reduce chrome salts (oxidation of catecholamines) which causes what?

A

A brown colour reaction

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18
Q

How are tumour cells of a phaeochromocytoma classically arranged?

A

In a trabecular pattern (nests)

19
Q

What do phaeochromocytomas secrete?

A

Catecholamines

20
Q

Give 6 symptoms associated with excess catecholamines?

A

Hyperglycaemia, sweating, palpitations, headache, anxiety, weight loss

21
Q

What is the classic triad of symptoms for phaeochromocytoma?

A

Hypertension, headache, sweating

22
Q

Phaeochromocytoma is a rare cause of what?

A

Secondary hypertension

23
Q

What are some clues for a possible diagnosis of phaechromocytoma?

A

Labile hypertension, postural hypotension, paroxysmal symptoms

24
Q

What is the onset of phaeochromocytoma?

A

Insidious

25
Q

Why is it so important to diagnose phaeochromocytoma?

A

They are a curable cause of hypertension and can be life threatening due to arrhythmias or ischaemia. There are also surgical and pregnancy related risks.

26
Q

Give 5 biochemical abnormalities of phaeochromocytoma?

A

Hyperglycaemia, mild hypercalcaemia, lactic acidosis, hypokalaemia, raised haemoglobin

27
Q

What are 4 clinical signs of complications of phaeochromocytoma?

A

LVF, myocardial necrosis, stroke, shock paralytic bowel

28
Q

What are the two steps to diagnosing phaeochromocytoma?

A

Confirm catecholamine excess, identify source of catecholamine excess

29
Q

How do you confirm catecholamine excess?

A

2 x 24 hour urinary catecholamines, plasma catecholamines at time of symptoms

30
Q

How do you find the source of excess catecholamines?

A

MRI abdomen/whole body, PET scan

31
Q

What is the main pharmacological management of catecholamine excess?

A

Alpha and beta blockers

32
Q

Should surgery be performed for phaeochromocytoma?

A

Yes, total excision where possible if not then tumour debulking

33
Q

What is the treatment if phaeochromocytoma is malignant?

A

Chemotherapy

34
Q

What is an important social test to take place if someone is diagnosed with phaeochromocytoma?

A

Genetic testing and family tracing

35
Q

What genetic conditions can phaeochromocytoma be associated with?

A

MEN2, Von-Hippel-Lindau, succinate dehydrogenase, NF, TS

36
Q

What condition can be known as Simple syndrome?

A

MEN2A

37
Q

What can occur in MEN2A?

A

Phaeochromocytoma, medullary thyroid carcinoma, parathyroid hyperplasia

38
Q

Describe the phaeochromocytoma which occurs in MEN2A?

A

Extra-adrenal and bilateral

39
Q

What genetic factor is MEN2A linked to?

A

Gain of function mutation in the RET oncogene on chromosome 10

40
Q

What can occur in MEN2B?

A

Phaeochromocytoma, medullary thyroid carcinoma, neuromas, ganglions, marfanoid habitus

41
Q

MEN2B is linked to a mutation in what?

A

RET oncogene

42
Q

What would make you suspicious of a phaeochromocytoma which was familial?

A

Younger patient and bilateral

43
Q

Phaeochromocytomas are more often malignant if associated with what?

A

Germline mutations of B subunit of succinate dehydrogenase

Endocrine- Adrenal (4 decks)

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