Adrenal 2

Question 1

How are the adrenal glands and gonads related?

Answer 1

they orginiated from the same tissue embryologically

they have very similar functions

Question 2

What are the layers of the adrenal coatex and what to they produce?

Answer 2

Zona glomerulosa = aldosterone
Zona fasciculata = cortisol
Zona reticularis = DHEA

Question 3

What is congenital adrenal hyperplasia?
What enzyme is deficient in 95% of cases?
What does this lead to?

Answer 3

• block on adrenal steroidogenesis affecting glucocorticoid synthesis
• 21-hydroxylase deficiency
• block in cortisol production via CYP21
• also decreases aldosterone production
• no negative feedback so massive increase in ACTH
• huge increase in DHEA –> androgens

Question 4

How can CAH be diagnosed?

Which population is more likely to be diagnosed late?

Answer 4

diagnosed by presence of 17hydroxyprogesterone (17OHP) in the blood and this is the last precursor before the blockade.
More likely to be identified in females due to ambiguous genitalia, males more likely to be diagnosed 14 days postnatally when mothers steroids are depleted and they have a salt wasting crisis

Question 5

What is the scale used to show stages of virilisation?

What are the issues with the later stages of virilisation?

Answer 5

Prader Stages I-V, V apparent normal male genitalia

• monthly blood in urine
• or pain as menses has no where to leave uterus

Question 6

What are 46XX males?

Answer 6

females who are assigned male at birth due to excessive androgen exposure in utero
- unusual now as neonatal screening programmes to pick up CAH

Question 7

What is the treatment of CAH?

How does it work?

Answer 7

glucocorticoid therapy

replaces low cortisol levels but also surpasses release of ACTH which it turn lowers DHEA production

Question 8

What is the balance required in glucocorticoid therapy?

Answer 8

too much glucocorticoid = cushings

not enough glucocorticoid = adrenal crisis, androgen crisis, an ovulation, oligomenorrhoea

Question 9

What are the differential diagnoses for adrenal incidentaloma?

Answer 9

adrenal cell carcinoma
adrenal cell adenoma
phaeochromocytoma

Question 10

What are the 2 initial problems when a patient presents with an adrenal incidentaloma?

Answer 10

1. ACC is not detected early enough
2. adrenal imaging is sensitive but not very specific –> unnecessary surgery, need to initially established if the mass is malignant or benign

Question 11

What are the 2 things we need to find out about an incidentaloma?

Answer 11

is it malignant

it is producing hormone?

Question 12

How do you exclude cushings?

Answer 12

dexamethasone supression test

24h urinary free cortisol

Question 13

How do you exclude phaeochromocytoma?

Answer 13

plasma metanephrines (metabolites of catecholamines)

Question 14

How do you diagnose primary hyperaldosteronism?

Answer 14

increased BP, or decrease K+

look at renin (low) and aldosterone (high)

Question 15

What additional endocrine tests do you with an adrenal tumour?

Answer 15

DHEAS (larger tumours)

17OHP (if high, indicates CAH)

Question 16

What characteristics mean its more likely to be a malignant tumour and hence need surgery?

Answer 16

age - younger, more likely to be cancer 
tumour size - >4cm 33% malignancy 
imaging morphology - irregular mass, inhomogeneous 
CT - density >20 HU
MRI - irregular enhancement

Question 17

What tumour types can imaging not differentiate between?

Answer 17

ACC and phaeochromocytoma

Question 18

What are hounsfield units?

Answer 18

measure of density
dense <10 –> likely to be adenoma
higher HU >20 –> likely to be carcinoma

Question 19

What is phaeochromocytoma?

Answer 19

tumour of chromaffin tissue secreting noradrenalin, thus located in the adrenal medulla
or paraganglioma = tumour of chromatin tissue in sympathetic ganglia

Question 20

What are the 4 genes which predispose to phaeochromocytoma?

Answer 20

RET protooncogene –> multiple endocrine neoplasia
VHL –> von hype lindau syndrome
NF-1 –> neurofibromatosis (von Recklinghausen)
SDHC –> familial head and neck paraglionoma

Question 21

What are the signs and symptoms of phaeochromocytoma?

Answer 21

• hypertension in 90% of cases
• headaches, sweating, palpitations, pallor, nausea, tremor, anxiety, postural hypotension (low plasma volume)
• supraventricular tachycardia
• myocardial infarction/ischaemia
• cardiomyopathy and heart failure
• fever, weight loss

Question 22

How is phaeochromocytoma diagnosed?

Answer 22

24h urinary metanephrines
24h urinary catecholamines
plasma total metanephrines
imaging

Question 23

What is the medical therapy for phaeochromocytoma?

What is important for surgical therapy?

Answer 23

Medical = alpha blockade (doxasosin), beta blockade if tachycardia (propranolol)
Surgery = never operate without alpha blockade, requires special anaesthesiology care

Question 24

What is the medical therapy for malignant phaeochromocytoma?

Answer 24

IMIBG therapy

DOTATOC therapy

Question 25

When would you do a fine needle aspiration?

Answer 25

ALMOST NEVER as puncture
only all if all the following criteria are met:
- history of extra-adrenal malignancy
- HU>20 in non-contrast CT
- no evidence of phaechromocytoma
- outcome will affect therapeutic strategy

Question 26

What is the treatment of choice for ACC?

Answer 26

• complete tumour removal by an expert surgeon using open surgery

Question 27

What is adjuvant therapy in ACC?

Answer 27

give mitotane post surgery

this is an adrenal toxic that destroys the last remaining cells and reduces recurrence and metastasis