Adrenal Disorders Flashcards
What is an adrenal adenoma
benign cancer emerging from the cells of the adrenal cortex
Associations with adrenal adenoma
Increase in frequency with age
Histological features of an adrenal adenoma
Well differentiated small, solitary lesions
Bright yellow and buried within the gland
How do patients usually get a diagnosis of adrenal adenoma
Often an incidental finding during abdominal imaging
How do patients with hyperfunctioning adrenal adenoma present
Excess hormone secretion e.g. cushings, conn syndrome
Investigations for adrenal adenoma
CT, MRI
hormonal testing
Management of adrenal adenoma without biochemical abnormality
Can be left in situ
When may a surgical excision of an adrenal adenoma be required
Lesion is functioning
Lesion is large - can be considered potentially malignant
What is an adrenocortical carcinoma
Very rare malignancy of the adrenal cortex
What is adrenocortical carcinoma associated with in young patients
Li-fraumeni syndrome
Histological features of an adrenocortical carcinoma
Large
Haemorrhage and necrosis
Capsular or vascular invasion
Local invasion of adrenocortical carcinoma
Retroperitoneum
Kidney
Metastasis of adrenocortical carcinoma
Usually haematogenous to the liver, lung and bone
Clinical presentation of adrenocortical carcinoma
Hormonal effects, abdominal mass effects
Carcinomas with necrosis can cause fever
Imaging of adrenocortical carcinoma
CT or MRI
Management of adrenocortical carcinoma
Resection with adjuvant therapy
What is neuroblastoma
Malignant neuroendocrine tumour of the sympathetic nervous system
Where does a neuroblastoma originate from
Neural crest cells
Mean age of diagnosis of a neuroblastoma
23 months
Symptoms of neuroblastoma
Loss of appetite
Vomiting
Weight loss
Fatigue
Bone pain
Bloods in neuroblastoma
Anaemia, raised inflammatory markers, abnormal coag
Investigations for neuroblastoma
Bloods, biopsy, CT
Management of neuroblastoma in low risk patients
Observed for spontaneous resolution or local resection
Management of neuroblastoma in high risk patients
Multi-agent chemotherapy, surgery and radiotherapy
What is a pheochromocytoma
Catecholamine secreting tumour
What is a pheochromocytoma typically derived from
Chromaffin cells of the adrenal medulla
What is pheochromocytoma known as
The 10% cancer
10% rule of pheochromocytoma
10% bilateral, malignant, outside of the adrenal gland
Name some genetic disorders associated with pheochromocytoma
MEN2
neurofibromatosis type 1
Von hippel-Lindau disease
Classic presentation of pheochromocytoma
Triad of hypertension, headache and sweating
Name some signs of complications of pheochromocytoma
LV failure, stroke, paralytic ileus of bowel, myocardial necrosis
2 initial tests of pheochromocytoma
Plasma free metanephrines
24 hour urine catecholamines
Imaging used in pheochromocytoma
CT or MRI
Management of pheochromocytoma
Alpha blockers
THEN beta blockers
THEN surgical removal
What is congenital adrenal hyperplasia
Inherited group of disorders with a deficiency in one of the enzymes necessary for cortisol synthesis
Inheritance in congenital adrenal hyperplasia
Autosomal recessive 21a-hydroxylase deficiency
Pathophysiology of congenital adrenal hyperplasia
21a-hydroxylase deficiency prevents the production of aldosterone and cortisol
Increased testosterone
Reduced cortisol stimulates ACTH release and cortical hyperplasia
What causes non-classical congenital adrenal hyperplasia
A partial 21a-dehydroxylase deficiency
When does non-classic congenital adrenal hyperplasia present
Adolescence/adulthood
How does congenital adrenal hyperplasia present
Precocious puberty
Hirtuism
Acne
Oligomenorrhoea, infertility or sub-fertility
When does classic congenital adrenal hyperplasia present
At birth or just after
How does classic congenital adrenal hyperplasia present
Genital ambiguity in females
Adrenal failure - biochemical pattern of Addisons disease
Investigations of congenital adrenal hyperplasia
Basal 17-OH progesterone
Genetic analysis
Paediatric management of congenital adrenal hyperplasia
Glucocorticoid replacement
Surgical correction
Mineralocorticoid replacement in some patients
Adult management of congenital adrenal hyperplasia
Control androgen excess
Glucocorticoid replacement
Restore fertility
What is adrenal crisis
Acute, severe glucocorticoid deficiency
What causes adrenal crisis
Stress in a patient with underlying adrenal insufficiency
OR
sudden discontinuation of glucocorticoids after prolonged therapy
Clinical presentation of adrenal crisis
Stress
Vomiting
Abdominal pain
Hypotension
Shock
Management of adrenal crisis
Rehydration: rapid bolus then 0.9% sodium chloride
Steroids: 100mg hydrocortisone IV injection then 24 hour infusion OR IM injection every 6 hours
What is Cushing’s syndrome
Increased free circulating glucocorticoid (cortisol)
What is cushings disease
A pituitary adenoma secreting excess ACTH which causes excess cortisol release from the adrenal glands
Who commonly presents with Cushing’s syndrome
Women aged 20-40
What is the most common cause of cortisol excess
Administration of synthetic steroids
Name the main causes of ACTH dependent Cushing’s syndrome
Cushing’s disease
Carcinoma of the lungs or pancreas
Name the main causes of ACTH independent Cushing’s syndrome
Exogenous steroids
Adrenal tumour
Adrenal cortical nodular hyperplasia
False positive: severe depression, severe alcoholism
Adrenal enlargement in ACTH-independent Cushing’s syndrome
Usually nodular
Adrenal enlargement in ACTH-dependent Cushing’s syndrome
Usually diffuse
Name some consequences of increased cortisol levels
Protein loss
Altered carb and lipid metabolism
Excess mineralocorticoid
Excess androgen
Clinical features of Cushing’s syndrome
Moon face
Central obesity, stretch marks
Buffalo hump
Muscle wasting
Hirsutism
Hyperpigmentation in patients with Cushing’s disease
3 tests to establish cortisol excess
Overnight dexamethasone suppression test
24 hr urine free cortisol
Diurnal cortisol variation
Abnormal result for an overnight dexamethasone suppression test
> 130 nmol/l cortisol
Diagnostic test for Cushing’s syndrome
Low dose dexamethasone suppression test
How to determine the cause of Cushing’s syndrome
Serum ACTH
If low, adrenal imaging
If high, pituitary MRI
Drug management of Cushing’s syndrome
Metyrapone when other treatments fail or waiting for radiotherapy to work
Surgical management of Cushing’s syndrome
Trans-sphenoidal removal of pituitary tumour
Surgical removal of adrenal tumour OR ACTH-producing tumour elsewhere
What is hyperaldosteronism
High levels of aldosterone
What is conns syndrome
Adrenal adenoma producing too much aldosterone
What is primary hyperaldosteronism
Where the adrenal glands are directly responsible for producing too much aldosterone
Causes of primary hyperaldosteronism
Bilateral adrenal hyperplasia
Conns syndrome
Familial hyperaldosteronism
What is the most common cause of secondary hypertension
Primary hyperaldosteronism
Clinical presentation of primary hyperaldosteronism
Significant hypertension
Alkalosis
Hypokalaemia
Management of conns syndrome
Unilateral laparoscopic adrenalectomy
Management of bilateral adrenal hyperplasia
Spironolactone
What causes secondary hyperaldosteronism
Excessive renin stimulating the release of excessive aldosterone
Causes of secondary hyperaldosteronism
Renal artery stenosis
Heart failure
Liver cirrhosis and ascites
What is renal artery stenosis
Narrowing of the artery supplying the kidney
What causes renal artery stenosis usually
Atheroma
What can be the cause of renal artery stenosis in younger females
Fibromuscular dysplasia
Clinical presentation of secondary hyperaldosteronism
Hypertension
Investigation in hyperaldosteronism
Aldosterone : renin ratio
High aldosterone low renin indicates…
Primary aldosteronism
High aldosterone high renin indicates…
Secondary hyperaldosteronism
What can be used to investigate renal artery stenosis
Doppler US
CT angiogram
MRA - magnetic resonance angiography
Management of renal artery stenosis
Percutaneous renal artery angioplasty via the femoral vein
Management of secondary hyperaldosteronism
Spironolactone
What causes secondary adrenal insufficiency
Lack of production of ACTH by the pituitary gland
What causes tertiary adrenal insufficiency
Lack of CRH secretion by the hypothalamus
Clinical presentation of secondary and tertiary adrenal insufficiency
SIMILAR TO ADDISONS
no hypertension
Pale skin
Management of secondary and tertiary adrenal insufficiency
Hydrocortisone replacement
What’s another name for primary adrenal insufficiency
Addisons disease
Features of adrenal insufficiency in babies
Lethargy, vomiting, poor feeding, hypoglycaemia, jaundice, failure to thrive
Clinical presentation of Addisons disease
Skin pigmentation
Weakness, fatigue, N+V, weight-loss, dizziness and low BP, abdo pain
Biochemistry in Addisons disease
Low cortisol
Low ACTH
Low aldosterone
High renin
Biochemistry in secondary adrenal insufficiency
Low cortisol
Low ACTH
normal aldosterone
Normal renin
Test for adrenal insufficiency
Short synacthen test
Pharmacological management of Addisons disease
Hydrocortisone as cortisol replacement
Fludrocortisone as aldosterone replacement
Sick day rules in adrenal insufficiency
Increase steroid replacement when unwell or undergoing other stress
what is the most common cause of addisons
autoimmune adrenalitis
