21. Adrenal and Parathyroid Pathology Flashcards
What is the most common cause of cushing’s syndrome?
Exogenous steroids
What are the adrenal causes of cushing’s syndrome?
Adenoma/carcinoma
Nodular hyperplasia
What are the paraneoplastic causes of cushing’s syndrome?
Small cell
Carcinoid
Medullary of thyroid
Islet cell of pancreas
Why should care be taken when taking a patient off exogenous steroids?
Steroids cause adrenal glands to atrophy so they can’t meet the demand by themselves
What are the clinical features of Cushing’s syndrome?
Hypertension
Weight gain, moon face
Hyperglycaemia
Fragile skin, osteoporosis
What are the effects of hyperaldosteronism?
Hypernatraemia
Hypokalaemia
Hypertension
How can primary and secondary hyperaldosteronism be differentiated?
Primary: shows reduced plasma renin as suppressed RAAS
Renin increased in secondary
What are the causes of primary hyperaldosteronism?
Conn’s syndrome: adrenocortical adenoma
Adenocortical hyperplasia
Glycocorticoid-suppressible hyperaldosteronism
What is the treatment for hyperaldosteronism?
Surgically remove adenomas
Spironolactone if hyperplasia
Which hormones are produced in each part of the adrenal cortex?
Glucocorticoids= fasciculata
Mineralocorticoids= glomerulosa
sex hormones= reticularis
What are the causes of secondary hyperaldosteronism?
CCF
Renal artery stenosis
Low albumin
Pregnancy
What enzyme is typically deficient in adrenogenital disorders?
21-hydroxylase which usually converts progesterone to 11-deoxycorticosterone
What are the causes of an acute adrenal crisis?
Addison’s with an additional stressor
Rapid withdrawal from exogenous steroids
Massive adrenal haemorrhage
What are the symptoms of an acute adrenal crisis?
Vomiting
Abdominal pain
Low BP
Coma and collapse
What is Waterhouse Friedriechsen Syndrome?
Bilateral adrenal haemorrhage a/w neisseria meningitidis sepsis
What is Addison’s disease?
Progressive destruction of the adrenal cortex
What are the causes of Addison’s disease?
Autoimmune adrenalitis
Infections
Metastatic carcinoma
What are the clinical features of Addison’s disease?
Progressive weakness and fatigue
Anorexia, nausea, vomiting and weight loss
Hyperpigmentation of skin
High K+, low Na+, BP and glucose
What is the appearance of adenomas in the adrenal cortex?
Well circumscribed
Yellow due to lipid content
Haemorrhage, cystic degeneration, calcification
Where do adrenal carcinomas spread to?
Adrenal vein, vena cava and lymphatics
Mets to lungs
What proportion of phaeochromocytomas are familial?
1/4
MEN and von Hippel Lindau
What is a paraganglioma?
Phaeochromocytoma outside the adrenal gland, typically in the carotid bodies
What are the clinical features of a phaeochromocytoma?
Hypertension, tachycardia, palpitations, headaches, sweating, tremor, abdomen and chest pain
Catecholamine cardiomyopathy
How is a phaeochromocytoma diagnosed?
24 hour urine catecholamines
for VMA and metanephrines
What name is given to supporting cells in the medulla?
Sustentacular cells
What are the causes of hyperparathyroidism?
Adenoma
Hyperplasia
Carcinoma
What are the causes of secondary hyperparathyroidism?
Renal failure
Chronic low calcium
What are the causes of hypoparathyroidism?
Surgical removal in thyroidectomy
DiGeorge syndrome
Autoimmune against Ca++ sensing receptors in the gland
Familial hypoparathyroidism
What type of inheritance are MEN syndromes?
AD
Which gene is associated with MEN 1?
MEN 1 gene on chromosome 2
Which neoplasms are associated with MEN 1?
Parathyroid
Pituitary
Pancreatic islet cells
What gene is associated with MEN 2?
RET proto-oncogene
What neoplasms are associated with MEN 2A?
Medullary carcinoma of thyroid
Phaeochromocytoma
Parathyroid hyperplasia
Name a variant of MEN 2A?
Familial medullary thyroid cancer
What conditions are associated with MEN 2B?
Medullary carcinoma of the thyroid
Phaeochromocytoma
Extra-endocrine manifestations eg. Marfan
What treatment is suggested if a patient has a RET mutation?
Prophylactic thyroidectomy