Tumours

Question 1

Adrenocortical tumours usually affect who?

Answer 1

Adults

Question 2

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

Answer 2

Li-Fraumeni Syndrome which mutates p53

Question 3

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

Answer 3

Metastases

Question 4

Are adrenal adenomas differentiated or not?

Answer 4

They are differentiated

Question 5

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

Answer 5

Carcinoma

Question 6

Virilising tumours are more likely to be?

Answer 6

Malignant

Question 7

Local invasion of an adrenal carcinoma will usually affect where?

Answer 7

Kidneys and retroperitoneum

Question 8

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

Answer 8

Lungs, liver, bone

Question 9

What is the prognosis of adrenal carcinoma like?

Answer 9

Poor

Question 10

Who does neuroblastoma occur in?

Answer 10

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

Question 11

What predicts a poorer outcome in neuroblastoma?

Answer 11

Amplification of n-myc and expression of telomerase

Question 12

What is a phaeochromocytoma?

Answer 12

A neuroendocrine tumour derived from chromaffin cells of the adrenal medulla

Question 13

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

Answer 13

Paraganglioma

Question 14

What are the 10% rules with phaeochromocytoma?

Answer 14

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

Question 15

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

Answer 15

When they are familial

Question 16

Malignant phaeochromocytomas are most common when?

Answer 16

They are extra-adrenal

Question 17

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

Answer 17

A brown colour reaction

Question 18

How are tumour cells of a phaeochromocytoma classically arranged?

Answer 18

In a trabecular pattern (nests)

Question 19

What do phaeochromocytomas secrete?

Answer 19

Catecholamines

Question 20

Give 6 symptoms associated with excess catecholamines?

Answer 20

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

Question 21

What is the classic triad of symptoms for phaeochromocytoma?

Answer 21

Hypertension, headache, sweating

Question 22

Phaeochromocytoma is a rare cause of what?

Answer 22

Secondary hypertension

Question 23

What are some clues for a possible diagnosis of phaechromocytoma?

Answer 23

Labile hypertension, postural hypotension, paroxysmal symptoms

Question 24

What is the onset of phaeochromocytoma?

Answer 24

Insidious

Question 25

Why is it so important to diagnose phaeochromocytoma?

Answer 25

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.

Question 26

Give 5 biochemical abnormalities of phaeochromocytoma?

Answer 26

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

Question 27

What are 4 clinical signs of complications of phaeochromocytoma?

Answer 27

LVF, myocardial necrosis, stroke, shock paralytic bowel

Question 28

What are the two steps to diagnosing phaeochromocytoma?

Answer 28

Confirm catecholamine excess, identify source of catecholamine excess

Question 29

How do you confirm catecholamine excess?

Answer 29

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

Question 30

How do you find the source of excess catecholamines?

Answer 30

MRI abdomen/whole body, PET scan

Question 31

What is the main pharmacological management of catecholamine excess?

Answer 31

Alpha and beta blockers

Question 32

Should surgery be performed for phaeochromocytoma?

Answer 32

Yes, total excision where possible if not then tumour debulking

Question 33

What is the treatment if phaeochromocytoma is malignant?

Answer 33

Chemotherapy

Question 34

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

Answer 34

Genetic testing and family tracing

Question 35

What genetic conditions can phaeochromocytoma be associated with?

Answer 35

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

Question 36

What condition can be known as Simple syndrome?

Answer 36

MEN2A

Question 37

What can occur in MEN2A?

Answer 37

Phaeochromocytoma, medullary thyroid carcinoma, parathyroid hyperplasia

Question 38

Describe the phaeochromocytoma which occurs in MEN2A?

Answer 38

Extra-adrenal and bilateral

Question 39

What genetic factor is MEN2A linked to?

Answer 39

Gain of function mutation in the RET oncogene on chromosome 10

Question 40

What can occur in MEN2B?

Answer 40

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

Question 41

MEN2B is linked to a mutation in what?

Answer 41

RET oncogene

Question 42

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

Answer 42

Younger patient and bilateral

Question 43

Phaeochromocytomas are more often malignant if associated with what?

Answer 43

Germline mutations of B subunit of succinate dehydrogenase