Pathology of the Adrenal Glands Flashcards Preview

Endocrine > Pathology of the Adrenal Glands > Flashcards

Flashcards in Pathology of the Adrenal Glands Deck (44):
1

Briefly describe how congenital problems can cause adrenocortical hyperplasia?

Group of autosomal recessive disorders
Deficiency/ lack of enzyme required for biosynthesis
Altered biosynthesis leads to increased androgen production
Reduced cortisol stimulates ACTH release and cortical hyperplasia

2

What are the 2 causes of endogenous ACTH production in acquired adrenocortical hyperplasia?

Pituitary adenoma (Cushing's disease)
Ectopic ACTH (paraneoplastic syndrome - SCLC)

3

With adrenocortical hyperplasia, is the adrenal gland enlargement diffuse or nodular?

Could be either
Diffuse - ACTH driven
Nodular - usually ACTH independent

4

What is the most common presentation of adrenocortical tumours?

Adults M=F
Incidental finding
Hormonal effects
Mass lesion
Carcinoma with necrosis can cause fever

5

What do adrenocortical adenomas look like?

Well circumscribed and differentiated
encapsulated lesions
2-3 cm
Yellow/ yellow brown surface
Small nuclei, rare mitoses

6

Are most adrenocortical adenomas functional or non-functional?

Most commonly non-functional but can be functional

7

Are most adrenocortical carcinomas more likely to be functional?

Yes

8

Are adrenocortical carcinomas common?

No - rare

9

Where do adrenocortical carcinomas spread?

Local invasion - retroperitoneum, kidney
Mets - usually vascular (liver, lung and bone)
Peritoneum and pleura
regional lymph nodes

10

Adrenocortical carcinomas and adenomas can be hard to tell apart. What features are suggestive of malignancy?

Large size (>50g, often >20cm)
Haemorrhage and necrosis
Frequent mitoses, atypical mitoses
lack of clear cells
capsular or vascular invasion

11

What is the only definite criteria for an adrenocortical carcinoma?

Metastatic spread

12

Define primary aldosteronism.

Autonomous production of aldosterone independent of it's regulators (ATII and K+)

13

How does primary aldosteronism present?

Significant hyperthyriodism
Hypokalaemia (~30% cases)
Alkalosis

14

What are the 4 subtypes of 1y aldosteronism?

Adrenal adenoma – Conn’s syndrome
Bilateral adrenal hyperplasia
Rare – genetic mutations and unilateral hyperplasia

15

How do you confirm aldosterone excess?

Measure Plasma aldosterone & renin (aldo:renin ratio)
Ratio >750 then Ix with saline suppression test
Failure of plasma aldosterone to suppress by >50% with 2L normal saline confirms 1y aldosteronism

16

How do you decide what the cause of the 1y aldosteronism is?

Confirm sub-type:
Adrenal CT – adenoma
Sometimes adrenal vein sampling to confirm adenoma is true source of aldosterone excess

17

How is bilateral adrenal hyperplasia managed?

Spironolactone or Epelerone (MR anatgonists)

18

How is 1y aldosteronism caused by an adrenal adenoma managed?

Unilateral laproscopic adrenalectomy - cures hypokalaemia and HTN in 30-70% cases

19

What is congenital adrenal hyperplasia?

Rare conditions associated with enzyme defects in the steroid pathway.

20

What is the most common enzyme defect associated with congenital adrenal hyperplasia?

21a-hydroxylase deficiency

21

Why is congenital adrenal hyperplasia a problem?

Altered biosynthesis leads to androgen excess. Decreased cortisol production stimulates ACTH release and cortical hyperplasia

22

What investigations are required for CAH?

Basal (or stimulated) 17-hydroxylase progesterone (key enzyme that helps us make cortisol and aldosterone)

23

What is the classical presentation of CAH?

males: poor weight gain
Adrenal insufficiency
Decreased Na+ but high potassium
Females: genital ambiguity

24

What S&S are seen in non-classical presentation of CAH?

hirsutes, acne, oligomenorrhoea, precocious puberty, infertility

25

What are the prinicples of treatment of CAH in a child?

Timely recognition required
Glucocorticoid replacement
Mineralocorticoid replacement (in some)
Surgical correction of ambiguous genitalia
Achieve maximal growth

26

If CAH is realised in an adult, what treatment principles are required?

Control androgen excess
Restore fertility
Avoid steroid over-replacement

27

What are acquired forms of adrenocortical hyperplasia?

Endogenous ACTH production (ituitary adenoma or ectopic ACTH from other tumour: paraneoplastic syndrome)
Bilateral adrena enlargement

28

The 2 forms of adrenocrotical hyperplasia are diffuse and nodular. What differentiates them?

Diffuse: ACTH driven
Nodular: ACTH independent

29

What can cause secondary adrenalcortical hypofunction?

Failure to stimulate adrenal cortex (hypothamalic disorder)
Suppression of adrenal cortex

30

What can cause acute primary adrenalcortical hypofunction?

Rapid withdrawl of steroid treatment
Adrenal crisis in pts with chronic adrenocortical insufficiecy
Massive adrenal haemorrhage

31

How is adrenalcortical insufficiency treated?

Hydrocortisone
Fludrocortisone
Education

32

What test is used to diagnose adrenalcortical insufficiency?

Short Synacthen test

33

What colour are phaeochromocytomas typically described as?

Tan

34

Which cells are phaeochormocytomas derived from?

Chromaffin cells of adrenal medulla

35

What do phaeochromocytomas secrete?

Cathecholamines

36

What scan can detect phaeochromocytomas and neuroblastomas?

MIBG scan

37

How are phaeochromocytomas treated?

Surgical excision
HTN treatment
Full alpha and beta-blockade - Phenoxybenzamine and Atenolol
Fluid and/or blood replacement

38

Describe both normal and results showing adrenal insufficiency from a short synacthen test.

Measure plasma cortisol before and 30 mins after IV ACTH injection
Normal: baseline >250nol/L Post-ACTH >480nmol/L

Addisons – baseline is very low Post-ACTH rise is very high

39

What are the biochemical abnormalities associated with phaeochormocytoma?

Hyperglycaemia
Decreased K+ (maybe)
High haemotocrit
Mild hypercalcaemia
lactic acidosis - in absence of shock

40

In the case of phaeochromocytoma, who should be investigated?

Family members with syndromes
resistant HTN
<50yo with HTN
Classical symptoms
Consider Ix if HTN and hyperglycaemia

41

What are the classical symtpoms of phaeochromocytoma?

“dreadful” “feeling of impending doom”
Pallor
Anxiety constipation
Weight loss abdominal pain
Pyrexia and sweating
Hypertension postural hypotension
Headache palpitations

42

Why are phaeochromocytomas known as the 10% tumour?

10% exra-adrenal
10% bilateral
10% malignant
10% not associated with HTN
10-25% familial

43

How is MEN inherited?

Autosomal doinant genetic condition

44

Briefly , what is Von-Hippel-Lindau syndrome?

Mutation in HIF 1-alpha
Autosomal dominant
Range of ascular tumours
Family screening vital